Carcinoma escamoso variedad sarcomatoide de lengua. Reporte de caso / Sarcomatoid variant squamous cell carcinoma of the tongue. Case report

Introducción. El carcinoma de células escamosas es una patología relativamente frecuente en Paraguay, que debe ser diagnosticada y tratada a tiempo. La variedad sarcomatoide es un subtipo poco frecuente, pero mucho más agresivo que la presentación convencional, con altas tasas de recurrencia y metástasis linfática. La exposición previa a radiación es uno de los principales factores desencadenantes. Caso clínico. Paciente de 83 años con antecedente de radioterapia por carcinoma escamocelular del paladar blando, quien consultó por una masa en el borde lateral de la lengua que correspondió a un carcinoma escamocelular del subtipo sarcomatoide. Resultados. El paciente fue sometido a cirugía y quimioterapia, pero presentó recaída tumoral a los cuatro meses, sin aceptar una cirugía de rescate, optando por el tratamiento paliativo y falleciendo a los pocos meses. Conclusión. El examen exhaustivo de la cavidad oral en una primera consulta permite identificar lesiones en estadios tempranos y el tratamiento multidisciplinario temprano puede mejorar la supervivencia global. El pronóstico de estos pacientes en estadios avanzados es desalentador. Actualmente la cirugía microvascular es la mejor opción terapéutica, pero la hemiglosectomía sin reconstrucción sigue siendo una opción aceptable en nuestro medio, conociendo los altos costos de la primera y el requerimiento de un grupo mayor de especialistas, largos tiempos quirúrgicos y estancias hospitalarias.

Introduction. Squamous cell carcinoma is a relatively common pathology in Paraguay, which must be diagnosed and treated on time. The sarcomatoid variety is a rare subtype, but much more aggressive than the conventional presentation, with high rates of recurrence and lymphatic metastasis. Previous exposure to radiation is one of the main triggering factors. Clinical case. An 83-year-old patient with a history of radiotherapy for squamous cell carcinoma of the soft palate, who consulted for a mass on the lateral edge of the tongue that corresponded to a squamous cell carcinoma of the sarcomatoid subtype. Results. The patient underwent surgery and chemotherapy, but had tumor relapse after four months, without accepting salvage surgery, opting for palliative treatment and dying a few months later. Conclusion. Exhaustive examination of the oral cavity in a first consultation allows lesions to be identified in early stages and early multidisciplinary treatment can improve overall survival. The prognosis of these patients in advanced stages is discouraging. Currently, microvascular surgery is the best therapeutic option, but hemiglossectomy without reconstruction continues to be an acceptable option in our environment, knowing the high costs of the former and the requirement for a larger group of specialists, long surgical times and hospital stays.

SRF-rearranged cellular perivascular myoid tumor: a clinicopathological analysis of two cases / 中华病理学杂志

Objective: To investigate the clinicopathological features, immunophenotype, diagnosis and differential diagnosis of SRF-rearranged cellular perivascular myoid tumor. Methods: Two cases of SRF-rearranged cellular perivascular myoid tumor diagnosed in the Department of Pathology, Fudan University Shanghai Cancer Center from October 2021 to March 2022 were collected. Immunohistochemical staining, fluorescence in-situ hybridization (FISH) and next-generation sequencing (NGS) were performed, and the literature was reviewed. Results: Case 1, a 3-month-old boy presented with a painless tumor of the scalp, measuring about 2 cm in diameter. Case 2, a 3-year-old girl complained with a painless tumor of the knee, measuring approximately 1.5 cm in diameter. Microscopically, the tumor had a clear boundary and showed multinodular growth. The tumor was mainly composed of spindle cells arranged in long intersecting fascicles associated with thin, slit-like or branching ectatic vessels, focally forming hemangiopericytoma-like appearance. The tumor cells were abundant, but there was no obvious atypia. Mitotic figures (3-4/10 HPF) were noted. H-caldesmon and SMA were positive in both cases. Case 1 showed diffuse and strong positivity for Desmin, and focally for CKpan. Ki-67 proliferation index was 20% and 30%, respectively. FISH displayed NCOA2 gene translocation in case 1 and the RELA gene translocation in case 2. NGS detected the SRF-NCOA2 gene fusion in case 1 and the SRF-RELA gene fusion in case 2. Both patients underwent local excisions. During the follow-up of 5-14 months, case 1 had no local recurrence, while case 2 developed local recurrence 1 year post operatively. Conclusions: SRF-rearranged cellular perivascular myoid tumor is a novel variant of perivascular cell tumor, which tends to occur in children and adolescents. The tumor forms a broad morphologic spectrum ranging from a pericytic pattern to a myoid pattern, and include hybrid tumors with a mixture of pericytic and myoid patterns. Due to its diffuse hypercellularity and increased mitotic figures and smooth muscle-like immunophenotype, the tumor is easy to be misdiagnosed as myogenic sarcomas. The tumor usually pursues a benign clinical course and rare cases may locally recur.

Progress in the treatment of soft tissue sarcoma in the 2023 annual meeting of the American Society of Clinical Oncology / 中华肿瘤杂志

Soft tissue sarcoma (STS) is a group of rare malignant tumors originating from mesenchymal tissue, with a high degree of malignancy and a wide range of pathological subtypes. The prognosis varies among different subtypes, and treatment increasingly relies on selecting appropriate treatment methods for different subtypes. Surgical treatment is still the main treatment method at present, and the development of immune and targeted therapy also brings new hope for the treatment of soft tissue sarcoma. Immune checkpoint inhibitors, oncolytic viruses and T cell therapy have shown well safety and efficacy in clinical trials. Targeted drugs such as trabectedin and lenvatinib have changed the treatment pattern of soft tissue sarcoma. Currently, chemotherapy based on doxorubicin and ifosfamide is still the first line treatment for patients with advanced soft tissue sarcoma who have distant metastasis. However, the adverse reactions of doxorubicin limit its application in elderly patients, and trofosfamide has shown good efficacy and safety as an alternative in clinical trials. The efficacy of postoperative radiotherapy has been confirmed, which can reduce the local recurrence rate after surgical resection of soft tissue sarcoma. In summary, multimodal comprehensive treatment has become the main strategy for the treatment of soft tissue sarcoma. The combination of different treatment methods can generate synergistic effects and help patients obtain more clinical benefits, such as the combination of doxorubicin and immune checkpoint inhibitors, and the combination of antiangiogenic drugs and chemotherapy drugs. At the 2023 annual meeting of the American Society of Clinical Oncology (ASCO), oncologists from all over the world reported many researches related to the treatment of soft tissue sarcoma. This article aims to review the new progress in the treatment of soft tissue sarcoma in the 2023 annual meeting of ASCO.

Progress in the treatment of soft tissue sarcoma in the 2023 annual meeting of the American Society of Clinical Oncology / 中华肿瘤杂志

Soft tissue sarcoma (STS) is a group of rare malignant tumors originating from mesenchymal tissue, with a high degree of malignancy and a wide range of pathological subtypes. The prognosis varies among different subtypes, and treatment increasingly relies on selecting appropriate treatment methods for different subtypes. Surgical treatment is still the main treatment method at present, and the development of immune and targeted therapy also brings new hope for the treatment of soft tissue sarcoma. Immune checkpoint inhibitors, oncolytic viruses and T cell therapy have shown well safety and efficacy in clinical trials. Targeted drugs such as trabectedin and lenvatinib have changed the treatment pattern of soft tissue sarcoma. Currently, chemotherapy based on doxorubicin and ifosfamide is still the first line treatment for patients with advanced soft tissue sarcoma who have distant metastasis. However, the adverse reactions of doxorubicin limit its application in elderly patients, and trofosfamide has shown good efficacy and safety as an alternative in clinical trials. The efficacy of postoperative radiotherapy has been confirmed, which can reduce the local recurrence rate after surgical resection of soft tissue sarcoma. In summary, multimodal comprehensive treatment has become the main strategy for the treatment of soft tissue sarcoma. The combination of different treatment methods can generate synergistic effects and help patients obtain more clinical benefits, such as the combination of doxorubicin and immune checkpoint inhibitors, and the combination of antiangiogenic drugs and chemotherapy drugs. At the 2023 annual meeting of the American Society of Clinical Oncology (ASCO), oncologists from all over the world reported many researches related to the treatment of soft tissue sarcoma. This article aims to review the new progress in the treatment of soft tissue sarcoma in the 2023 annual meeting of ASCO.

Synovial sarcoma of the Hypopharynx in a Filipino female: A case report

@#Synovial sarcoma of the hypopharynx is an uncommon malignancy, with less than 100 cases reported in available journals. We report a case of a 22-year-old female presenting with dysphagia and enlarging hypopharyngeal mass, clinically diagnosed as hypopharyngeal malignancy, right, at least stage III. Histopathologic examination including immunohistochemistry study with TLE1 and SS18 Fluorescence In Situ Hybridization (FISH) confirm the diagnosis of synovial sarcoma. This is the first reported case of synovial sarcoma of the hypopharynx in the Philippines confirmed by SS18 FISH. Due to the size of the mass, chemoradiotherapy followed by surgery is the current plan of management for this patient.

Tratamiento multidisciplinario de un sarcoma recurrente de la fosa isquiorrectal / A successful multidisciplinary approach to a recurrent ischiorectal fossa sarcoma

Los tumores de la fosa isquiorrectal son poco frecuentes, habitualmente descritos en reportes o series de casos. Estas lesiones son un reto terapéutico, requiriendo un estudio preoperatorio apropiado, además de discusión y manejo guiado por un comité multidisciplinario, que permiten lograr resultados óptimos tanto oncológicos como funcionales. Presentamos un caso de un hombre de 73 años con antecedentes de resección de un tumor glúteo izquierdo en otro centro 5 años antes. La biopsia fue compatible con un sarcoma epiteloideo (SE) de alto grado, con margen quirúrgico < 0,5 mm que requirió ampliación de los márgenes posteriormente. Además, se realizó radioterapia adyuvante. Al cuarto año de seguimiento el paciente desarrolla dolor e induración con retracción en relación a cicatriz quirúrgica, siendo objetivada una recidiva tumoral local. Luego de una discusión multidisciplinaria, se realizó una desfuncionalización con ileostomía en asa laparoscópica y resección tumoral con preservación del ano y del piso pélvico. El defecto fue cubierto por el equipo de cirugía plástica utilizando un colgajo perforante de la arteria glútea superior. La biopsia confirmó la recidiva tumoral y los márgenes quirúrgicos fueron negativos. El paciente es dado de alta a los 25 días postoperatorios por cuidados del colgajo, sin complicaciones. Al año de seguimiento el paciente no presenta recidiva tumoral, la ileostomía fue cerrada, y sus resultados funcionales en términos defecatorios y de la herida son buenos.

Ischiorectal fossa tumors are rare lesions, mostly described in case reports or case series. These lesions represent a diagnostic and therapeutic challenge. Hence, an appropriate preoperative study and multidisciplinary discussion are essential to achieve good oncologic and functional results. We report a case of a 73-year-old male operated on five years before in another health center due to the diagnosis of a left gluteal tumor. The lesion was excised, and biopsies confirmed a high-grade epithelioid sarcoma with a close margin, requiring a subsequent wider excision of the surgical margins. The patient received adjuvant radiotherapy. After four years of follow-up, the patient developed mild pain with skin retraction around the former incision. A local recurrence was diagnosed by imaging. In a multidisciplinary team meeting, a decision to resect the lesion with preservation of the anus and the pelvic floor was taken. The patient underwent a laparoscopic defunctioning loop ileostomy and a resection of the recurrent tumor in the ischiorectal fossa with preservation of the anal sphincter. The defect was covered utilizing a superior gluteal artery perforator flap and a partial gluteus maximus muscle rotation. The tumor was completely excised with negative margins. The patient was discharged without complications after 25 days due to flap management. After one year of follow-up, the patient is recurrence-free, and the ileostomy was closed.

Sarcoma de la mama / Sarcoma of the Breast

Introducción: El sarcoma primitivo de la mama es el tumor maligno no epitelial menos frecuente. Se trata de una entidad infrecuente que presenta una incidencia menor al 1 por ciento de las neoplasias malignas de la mama y menos del 5 por ciento de todos los sarcomas. Objetivo: Presentar una serie de casos con diagnóstico de sarcoma de la mama por la baja incidencia de esta enfermedad a nivel mundial, su agresividad y su curso precario. Presentación de caso: Se presentan siete pacientes con diagnóstico de sarcoma de la mama, de las cuales seis correspondieron al sexo femenino; la edad promedio fue de 52,8 años. Desde el punto de vista anatomopatológico el diagnóstico fue heterogéneo pues se encontraron: tumor phylodes maligno, fibrosarcomas, sarcoma epitelioide, sarcoma pleomórfico y sarcoma de células claras. Conclusiones: El sarcoma de la mama es una entidad rara y poco frecuente(AU)

Introduction: Primitive sarcoma of the breast is the least frequent nonepithelial malignant tumor. It is a rare entity whose incidence accounts for less than 1 percent of malignant neoplasms of the breast and less than 5 percent of all sarcomas. Objective: To present a series of cases diagnosed with sarcoma of the breast due to the low incidence of this disease worldwide, as well as its aggressiveness and precarious course. Case presentation: Seven patients with a diagnosis of sarcoma of the breast are presented, six of whom were female. Their average age was 52.8 years. From the anatomopathological point of view, the diagnosis was heterogeneous, since malignant phyllodes tumor, fibrosarcomas, epithelioid sarcoma, pleomorphic sarcoma and clear cell sarcoma were found. Conclusions: Sarcoma of the breast is a rare and infrequent entity(AU)

Update in Imaging Evaluation of Bone and Soft Tissue Sarcomas / Atualização na avaliação por imagens dos sarcomas ósseos e das partes moles

Abstract The evolution in imaging evaluation of musculoskeletal sarcomas contributed to a significant improvement in the prognosis and survival of patients with these neoplasms. The precise characterization of these lesions, using the most appropriate imaging modalities to each clinical condition presented, is of paramount importance in the design of the therapeutic approach to be instituted, with a direct impact on clinical outcomes. The present article seeks to update the reader regarding imaging methodologies in the context of local and systemic evaluation of bone sarcomas and soft tissues.

Resumo A evolução na avaliação por imagens dos sarcomas musculoesqueléticos contribuiu para melhora significativa no prognóstico e na sobrevida dos portadores destas neoplasias. A caracterização precisa destas lesões, mediante utilização das modalidades de imagem mais adequadas a cada condição clínica apresentada, é de suma importância no delineamento da abordagem terapêutica a ser instituída, com impacto direto sobre os desfechos clínicos. O presente artigo busca atualizar o leitor a propósito das metodologias de imagem no contexto da avaliação local e sistêmica dos sarcomas ósseos e das partes moles.

Angiosarcoma gastrointestinal multifocal, con compromiso extenso de estómago, intestino delgado y colon. Reporte de un caso / Multifocal gastrointestinal angiosarcoma, with extensive gastric, small intestine and colon involvement. A case report

Introducción. El angiosarcoma es una neoplasia vascular originada a nivel del endotelio, de baja frecuencia, conocido por su agresividad y crecimiento acelerado. Alcanza solo el 1 al 2 % del total de los sarcomas. La presentación gastrointestinal es infrecuente y su incidencia es poco conocida debido al escaso reporte de casos en la literatura. Suele identificarse en etapas avanzadas debido a la dificultad del diagnóstico histopatológico por sus características morfológicas, siendo necesario aplicar tinciones especiales o estudio inmunohistoquímico. Caso clínico. Por su interés y singularidad, presentamos el caso de un paciente masculino de 54 años, quien consultó con hemorragia digestiva profusa, anemización y requerimiento de terapia transfusional. Resultados. Durante el proceso diagnóstico, en la endoscopia encontraron diversas lesiones multifocales que se extendían por gran parte del tracto gastrointestinal. El estudio histopatológico mostró angiosarcoma gastrointestinal. Discusión. El angiosarcoma del tracto gastrointestinal es extremadamente infrecuente, de difícil diagnóstico y bajas posibilidades de manejo curativo, con opciones terapéuticas limitadas, lo que configura un mal pronóstico a corto plazo

Introduction. Angiosarcoma is a vascular neoplasm originating from endothelial cells, known for its aggressiveness, accelerated growth and reduced frequency. Reach only 1 to 2% of total sarcomas. Gastrointestinal presentation is extremely rare, the true incidence is poorly known, due to the limited reports of this entity in the literature. It is usually identified in advanced stages in view of the difficulty of the histopathological diagnosis, attributable to its morphological characteristics, being necessary to apply special stains or immunohistochemical study. Case report. Due to their interest and uniqueness, we present the case of a 54-year-old male patient, who presented with a profuse gastrointestinal bleeding, anemia, and requirement for transfusion therapy. Results. The endoscopy detected several multifocal lesions that extended most of the gastrointestinal tract. The histopathological study showed gastrointestinal angiosarcoma. Discussion. Angiosarcoma of the gastrointestinal tract is extremely uncommon, difficult to diagnose and has low possibilities of curative management, with limited therapeutic options, which configures a poor prognosis in the short term

Seis casos consecutivos de sarcoma intracraneal primario en niños en dos instituciones privadas de Lima durante la pandemia del COVID-19 / Six consecutive cases of primary intracranial sarcoma in children in two private institutions from Lima during COVID-19 pandemic

Objetivo: El presente artículo describe las características clínicas e imagenológicas de seis pacientes en edad pediátrica con diagnóstico de tumor cerebral atendidos en dos instituciones privadas de Lima durante la pandemia del COVID-19. Material y métodos: Estudio descriptivo de casos de tumor cerebral en pacientes menores de 18 años, atendidos en dos clínicas privadas de Lima, desde marzo 2020 a diciembre 2021. Resultados: Seis casos consecutivos de tumores intracraneales fueron detectados durante la mayor parte del primer año de la pandemia COVID-19 (periodo de octubre del 2020 a marzo del 2021), todos ellos con el diagnóstico de sarcoma intracraneal primario. Antes de la pandemia, el último sarcoma intracraneal primario en niños operado en las instituciones mencionadas ocurrió en junio del 2018 y fue el único caso de este tipo de tumor en el año. Conclusiones: Durante la pandemia de COVID-19, se encontró una inusual frecuencia de sarcoma intracraneal primario en niños en dos instituciones privadas de Lima.

SUMMARY Objective: The article describes the clinical and neuroimaging characteristics of six children with the diagnosis of brain tumor attended in two private institutions from Lima, during the COVID-19 pandemic. Material and methods: Descriptive study of cases of brain tumor in patients aged 18 and younger, followed at two private institutions in in Lima, from March 2020 to December 2021. Results: Six consecutive cases of intracranial tumors were detected during a large part of the COVID-19 pandemic's first year (October 2020 to March 2021), all of them with diagnosis of primary intracranial sarcoma. Before the pandemic, the last primary intracranial sarcoma in children, operated in the mentioned institutions, occurred in June 2018 and was the only case that year. Conclusions: During the COVID-19 pandemic, an unusual frequency of primary intracranial sarcoma in children was found in two private institutions from Lima.

The Asian Sarcoma Consortium sarcoma preceptorship program: A program evaluation study utilizing the kirkpatrick model (level 1 and 2)

Background@#Sarcomas, a heterogenous group of malignancies consisting of tumors, are rarely encountered in most general oncology clinics. As a result, most general oncologists have very little experience in dealing with these tumors and resulting in poorer treatment and survival outcomes. Having successful collaborative efforts in research, the Asian Sarcoma Consortium (ASC) pioneered a Sarcoma Preceptorship Program with its main goal of increasing awareness of the multidisciplinary approach in the management of sarcomas.@*Objective@#The objective of this study is to evaluate the ASC Sarcoma Preceptorship Program using the first 2 levels of the Kirkpatrick Evaluation Model: Level 1) Reaction – through using satisfaction surveys; and Level 2) Knowledge – focusing on the amount of information gained from the activity evaluated by test-retest method.@*Methods@#The 2-day preceptorship program held in Singapore utilized educational activities such as didactic lectures, multidisciplinary case discussions, and case presentations. The program was evaluated using: 1) pre-test and posttest; 2) satisfaction survey from ASC; and 3) satisfaction survey made by the sponsor, Novartis Pharma.@*Results@#The preceptorship program enrolled 30 participants, most from the Asia-Pacific region. The overall results from the satisfaction surveys were generally optimistic, with results showing high levels of satisfaction with regard to the time allotment, scope of topics, and how helpful each session was. Test results showed that participants scored lower in post-test as compared to the pre-test. Computation of the Coefficient of Test reliability showed that although the pre-test was highly reliable, this was not true of the post-test.@*Conclusions@#Based on the Level 1 evaluation, the program was successful in terms of both participant and faculty satisfaction. However, certain areas for improvement were identified and recommendations were made in order to effect improvements for future iterations of this preceptorship.

Efficacy and safety of Anlotinib in the treatment of advanced sarcoma / 中华肿瘤杂志

Objective: Patients with advanced sarcomas have a dismal prognosis with few effective therapies. The purpose of this study was to evaluate the efficacy and safety of anlotinib in the treatment of advanced sarcoma and to explore the relationship between adverse events (AEs) and efficacy. Methods: Data from 45 advanced sarcoma patients who received anlotinib monotherapy at Affiliated Cancer Hospital of Zhengzhou University between June 2018 and August 2021 were retrospectively analyzed. According to Response Evaluation Criteria In Solid Tumors (RECIST) Version 1.1, the objective remission rate (ORR) and disease control rate (DCR) were calculated, and the progression free survival (PFS) and treatment-related AEs were recorded and analyzed. Survival analysis was conducted using the Kaplan-Meier survival rates were compared using the Log rank test. Results: Forty patients were treated for more than 1.5 months and received efficacy evaluation. The ORR and DCR after 3 months were 7.5%(3/40) and 80.0%(32/40), respectively. The overall ORR was 2.5%(1/40), the total DCR was 27.5%(11/40), and the median progression-free survival (m-PFS) was 6.70 months; The m-PFS of alveolar soft tissue sarcoma (ASPS) was 10.27 months, which was significantly longer than that of other subtypes of sarcoma (P=0.048). In addition, the DCR of ASPS and synovial sarcoma (SS) was significantly better than that of osteosarcoma (P<0.05). The most common AEs were elevated thyroid stimulating hormone (17.8%, 8/45), anemia (15.6%, 7/45), fatigue (11.1%, 5/45). Five patients developed grade 3 AEs after treatment; The PFS of patients with hand-foot syndrome after treatment was significantly longer than that of patients without hand-foot syndrome (14.10 vs 6.00, P=0.024). Conclusions: The efficacy of anlotinib in the treatment of ASPS and SS is better than that of other subtypes. The PFS in the group with hand-foot syndrome was significantly longer than that of the group without hand-foot syndrome.

Efficacy and safety of Anlotinib in the treatment of advanced sarcoma / 中华肿瘤杂志

Objective: Patients with advanced sarcomas have a dismal prognosis with few effective therapies. The purpose of this study was to evaluate the efficacy and safety of anlotinib in the treatment of advanced sarcoma and to explore the relationship between adverse events (AEs) and efficacy. Methods: Data from 45 advanced sarcoma patients who received anlotinib monotherapy at Affiliated Cancer Hospital of Zhengzhou University between June 2018 and August 2021 were retrospectively analyzed. According to Response Evaluation Criteria In Solid Tumors (RECIST) Version 1.1, the objective remission rate (ORR) and disease control rate (DCR) were calculated, and the progression free survival (PFS) and treatment-related AEs were recorded and analyzed. Survival analysis was conducted using the Kaplan-Meier survival rates were compared using the Log rank test. Results: Forty patients were treated for more than 1.5 months and received efficacy evaluation. The ORR and DCR after 3 months were 7.5%(3/40) and 80.0%(32/40), respectively. The overall ORR was 2.5%(1/40), the total DCR was 27.5%(11/40), and the median progression-free survival (m-PFS) was 6.70 months; The m-PFS of alveolar soft tissue sarcoma (ASPS) was 10.27 months, which was significantly longer than that of other subtypes of sarcoma (P=0.048). In addition, the DCR of ASPS and synovial sarcoma (SS) was significantly better than that of osteosarcoma (P<0.05). The most common AEs were elevated thyroid stimulating hormone (17.8%, 8/45), anemia (15.6%, 7/45), fatigue (11.1%, 5/45). Five patients developed grade 3 AEs after treatment; The PFS of patients with hand-foot syndrome after treatment was significantly longer than that of patients without hand-foot syndrome (14.10 vs 6.00, P=0.024). Conclusions: The efficacy of anlotinib in the treatment of ASPS and SS is better than that of other subtypes. The PFS in the group with hand-foot syndrome was significantly longer than that of the group without hand-foot syndrome.

CIC-rearranged sarcoma: a clinicopathological analysis of four cases / 中华病理学杂志

Objective: To investigate the clinicopathological features and differential diagnosis of CIC-rearranged sarcoma (CRS). Methods: Five CRSs of 4 patients (2 biopsies of pelvic cavity and lung metastasis from case 4) diagnosed in the First Affiliated Hospital of Nanjing Medical University were enrolled from 2019 to 2021. All cases were evaluated by clinical presentation, H&E, immunohistochemical staining and molecular analysis and the related literature was reviewed. Results: There were one male and three females, the age at diagnosis ranged from 18 to 58 (mean 42.5) years. Three cases were from the deep soft tissues of the trunk and one case from the skin of foot. Grossly, the tumor size ranged from 1 to 16 cm. Microscopically, the tumor was arranged in nodules or solid sheets. The tumor cells were typically round or ovoid, with occasional spindled or epithelioid morphology. The nuclei were round to ovoid with vesicular chromatin and prominent nucleoli. Mitotic figures were brisk (>10/10 HPF). Rhabdoid cells were seen in four of five cases. Myxoid change and hemorrhage were observed in all samples and two cases showed geographic necrosis. Immunohistochemically, CD99 was variably positive in all samples, while WT1 and TLE-1 were positive in four of five samples. Molecular analysis showed CIC-rearrangements in all cases. Two patients succumbed within 3 months. One had mediastinal metastasis 9 months after surgery. One underwent adjuvant chemotherapy and remained tumor-free 10 months after diagnosis. Conclusions: CIC-rearranged sarcoma is uncommon and shows aggressive clinical course with dismal prognosis. The morphological and immunohistochemical characteristics can largely overlap with a variety of sarcomas; hence, knowledge of this entity is vital to avoid potential diagnostic pitfalls. Definitive diagnosis requires molecular confirmation of CIC-gene rearrangement.

Radiation-associated sarcomas of bone and soft tissue: a clinicopathological analysis of 46 cases / 中华病理学杂志

Objective: To investigate the clinical, imaging, histological, and molecular features and the differential diagnosis of radiation-associated sarcomas of bone and soft tissue. Methods: Forty-six cases of radiation-associated sarcomas of the bone and soft tissue in Beijing Jishuitan Hospital from January 2010 to January 2022 were retrospectively analyzed; and the imaging, histological features and immunophenotype were examined. Results: There were 33 females and 13 males, aged from 18 to 74 years, with a mean of 52 years. The most common site of radiation-associated sarcomas were the limbs and spine (15 cases), followed by the chest (9 cases). The primary diseases included epithelial tumors (15 breast cancer, 6 cervical cancer, and 5 bowel cancer), hematolymphoid tumors, bone and soft tissue tumors and infectious lesions. The latent period of radiation-associated sarcomas ranged from 2-22 years, with an average of 11.6 years. Histopathologically, the morphology was divergent from the primary tumor. The most common malignant tumor type was undifferentiated sarcoma (22 cases), followed by osteosarcoma (16 cases). The immunophenotype of radiation-related sarcoma was almost the same as the corresponding soft tissue sarcoma. Conclusions: Radiation-induced sarcoma has a wide range of primary tumor types and its imaging, morphology and immunohistochemical features are similar to those of the primary sarcoma of bone and soft tissue. Clinical correlation is often recommended for the differential diagnosis.

Sarcoma uterino de topografía cervical / Sarcoma of the uterus of cervical topography / Sarcoma de colo de utero

Introducción: Los sarcomas uterinos son tumores poco frecuentes. Se originan en el tejido uterino de origen mesodérmico (músculo, tejido conjuntivo o estroma), siendo los más frecuentes los leiomiosarcomas, seguidos de los sarcomas del estroma endometrial. La expresión clínica de la enfermedad puede ser con sangrados genital, distensión abdominal y/o síntomas compresivos. Muchas veces se diagnostican en pacientes que son hatadas quirúrgicamente con diagnóstico de miomatosis uterina y, lo más frecuente es el diagnostico anatomopatológico posto peratorio de la pieza quirúrgica. El rol de la cirugía (histerectomía y anexectomía) es fundamental en el tratamiento. Descripción: El video presenta la evaluación diagnóstica y el abordaje quirúrgico de una paciente en la que se diagnosticó un leiomiosarcoma uterino de topografía cervical. Conclusiones: Se trata de una patología poco frecuente, con la particularidad de la ubicación, generando dificultades diagnósticas (clínica y paraclínicas) así como de abordaje quirúrgico. Ver video en https://www.youtube.com/watch?v=9rr8R2L7CWs

Summary: Introduction: Sarcoma of uterus are rather unusual tumors. They ori ginate in the mesodermal tissue of the uterus (muscle, connective tissue or stroma), the most frequent ones being leiomyosarcoma, followed by endometrial stromal sarcomas. The clinical presentation of the disease may include genital bleeding, abdominal distention or compressive symptoms. They are often diagnosed in patients who are surgically treated with a diagnosis of uterine myoma, upon the postoperative pathological study of the surgical piece. The role of surgical treatment (hysterectomy and anexectomy) is of the essence. Description: The video presents the diagnostic assessment and sur gical approach of a patient who was diagnosed with leiomyosarcoma of the uterus with cervical topography. Conclusions: It it rather an unusual condition with a special loca tion. which results in diagnostic difficulties (both clini cal and paraclinical) as well as hi the surgical approach. Watch video at https://www.youtube.com/watch?v=9rr8R2L7CWs

Introdução: Os sarcomas uterinos sào tumores raros. Originam-se no tecido uterino de origem mesodérmica (músculo, tecido conjuntivo ou estróina), sendo os leiomiossarco- mas os mais frequentes, seguidos dos sarcomas estro- mais endometriais. A expressão clínica da doença pode ser com sangramento genital, distensão abdominal e/ou sintomas compressivos. São frequentemente diagnosticados em pacientes tratadas cirurgicamente com diagnóstico de miomatose uterina e, mais frequentemente, com diagnóstico da anatomia patológica pós-operatória da peça cirúrgica. O papel da cirurgia (histerectomia e anexectomia) é essencial no tratamento. Descrição: O vídeo apresenta a avaliação diagnóstica e abordagem cirúrgica de uma paciente diagnosticada com leiomiossarcoma de colo de útero. Conclusões: É uma patologia pouco frequente, pela particularidade da sua localização, gerando dificuldades de diagnóstico (clínico e complementar) bem como de abordagem cirúrgica. Veja o vídeo em https://www.youtube.com/watch?v=9rr8R2L7CWs

Determinación del grado de agresividad tumoral mediante técnicas de inteligencia artificial aplicadas a imágenes de resonancia magnética / Measuring tumor aggressiveness through artificial intelligence

Objetivo: Determinar el grado de agresividad tumoral mediante técnicas de inteligencia artificial utilizando imágenes de resonancia magnética de sarcomas con grado histológico comprobado. Materiales y Métodos: Dos cohortes retrospectivas independientes de pacientes con sarcomas de partes blandas. Para cada paciente de las dos cohortes se adquirieron tres tipos de secuencias de imágenes como indican los protocolos clínicos: potenciadas en T1, en T2 con supresión grasa (T2FS) y STIR. A fin de desarrollar el modelo de inteligencia artificial, se utilizaron 134 imágenes, tanto las de alto grado como las de bajo grado, en T1 y T2 tomando la imagen más representativa del tumor en cualquier corte. Esto se traduce en 36 millones de píxeles que serán analizados por el programa Landing AI. Resultados: La precisión promedio del modelo fue del 84,3% y la sensibilidad, del 73,3%, con un umbral de confianza de 0,66, lo que demuestra inicialmente que se obtuvo un modelo de buena calidad para predecir con imágenes de resonancia magnética el grado de agresividad de un sarcoma de partes blandas antes de la biopsia. Conclusiones: Se presenta un enfoque novedoso para abordar un tipo de enfermedad infrecuente usando técnicas de inteligencia artificial para determinar el grado tumoral en imágenes de resonancia magnética. Según los resultados de nuestro modelo, se lo puede considerar como una segunda opinión experta al realizar los estudios por imágenes antes de la biopsia. Nivel de Evidencia: III

Objective: To determine the degree of tumor aggressiveness by means of artificial intelligence techniques using magnetic resonance images of sarcomas with proven histological grade. Materials and Methods: Two independent cohorts of patients with soft tissue sarcomas (STS) were retrospectively collected. For each patient in the two cohorts, three types of imaging sequences were acquired as indicated by the clinical protocols: T1-weighted (T1), fat-suppressed T2-weighted (FST2) and STIR. For the development of the artificial intelligence model, 134 images were used, both high-grade and low-grade T1 and T2 images, taking the most representative image of the tumor at any slice. This translated into more than 36 million pixels that were analyzed by the Landing AI program. Results: To determine the degree of tumor aggressiveness by means of artificial intelligence techniques using magnetic resonance The model's average accuracy was 84.3%, and its sensitivity was 73.3%, with a confidence threshold of 0.66, indicating that a good quality model was generated for predicting the grade of aggressiveness of an STS prior to biopsy using MRI scans. Conclusions: A novel approach is presented to address a rare pathology using artificial intelligence techniques to determine the tumor grade based on nuclear magnetic resonance images. Based on the results of our model, it can be considered as a second expert opinion when performing imaging studies prior to biopsy. Level of Evidence: III

Papel da ressonância nuclear magnética na predição de grau histológico em Sarcomas de partes moles / Role of magnetic resonance imaging in the prediction of histological grade in soft tissue sarcomas

INTRODUÇÃO: Os sarcomas são tumores malignos raros e compreendem um grupo heterogêneo de tumores, com mais de 60 subtipos histológicos. A importância da classificação do grau histológico é fundamental para a definição da linha de tratamento a ser seguida. A busca por métodos que aumentem a acurácia na definição de alto grau deve ser constante. OBJETIVO: Avaliar o papel da Ressonância Nuclear Magnética na predição do grau histológico nos sarcomas de partes moles. MATERIAIS E MÉTODOS: Estudo observacional, transversal e retrospectivo. Critérios de inclusão foram pacientes com mais de 18 anos, submetidos a biópsia por agulha grossa guiada ou revisão de biópsia externa, exame de RNM e a ressecção do tumor primário no ACCCC. Exclusão de pacientes submetidos a biópsia cirúrgica. Principais variáveis foram: tamanho do tumor, idade, localização, subtipo histológico, grau histológico da biópsia e da peça cirúrgica ­ sendo utilizado classificação em alto grau (G2 e G3 da FNCLCC) e baixo grau (G1). Os critérios radiológicos da RNM (tamanho, definição das margens (regular ou irregular), heterogeneidade de sinal em T2 (maior 50% ou menor 50%), sinal de alta intensidade peritumoral em T2 (edema peritumoral presente ou ausente), realce pós-contraste peritumoral (presente ou ausente) foram avaliados por dois radiologistas da nossa instituição. Além disso, foi realizado o cálculo de um score para avaliação de predição do alto grau histológico, por meio da somatória dos critérios positivos analisados (considerando ser de 0 (zero), quando nenhum critério foi identificado a 5 (cinco) quando todos os critérios forem positivos). Utilizamos teste de associação Qui-quadrado entre os itens da RNM e o grau histológico da peça cirúrgica e, em seguida, utilizamos teste de regressão logística a fim de identificar a associação independente, de modo a avaliar o valor da RNM na predição do grau histológico. O nível de significância adotado foi de 5%. RESULTADOS: Sessenta e oito pacientes foram incluídos no estudo. Trinta mulheres e 38 homens. A biópsia identificou 54 tumores de alto grau e 14 tumores de baixo grau. Já na peça cirúrgica tivemos 52 tumores de alto grau e 16 de baixo grau. A via mais comum de biópsia foi biópsia por agulha grossa guiada por ultrassonografia com 54,4% dos casos. O subtipo mais comum foi o lipossarcoma com 17(25%) casos, seguido pelo sarcoma sinovial 17,6% e leiomiossarcoma 10,3%, assim como localização em extremidade com 64,7%. Identificamos uma maioria de tumores maiores que 5cm, correspondendo à 89,7%. A regularidade das margens 43 (63,2%) foram regular, e apenas 25 (36,8%) consideradas irregular. Já na avaliação da heterogeneidade tumoral em T2 foram 36 (52,9%) heterogêneos contra 32 (47,1%) com menos 50% de heterogeneidade. O sinal de alta intensidade peritumoral em T2 estava presente em 45 (66,2%) das vezes, ao passo que, o realce pós-contraste peritumoral estava presente em 31 (45,6%). O score ficou: dois com score 0 (zero), 10 com score 1 (um), 16 com score 2 (dois), 13 com score 3 (três), 18 com score 4 (quatro) e nove com score 5 (cinco). Os critérios da RNM que demonstraram associação com grau histológico foram sinal de alta intensidade peritumoral em T2 (p<0,001) e realce peritumoral pós-contraste (p=0,006). Na regressão logística a presença de sinal de alta intensidade peritumoral em T2 (OR 11,8) e o realce pós-contraste peritumoral (OR 8,8) também foram preditivos de tumor de alto grau. Apesar de os demais critérios da RNM não apresentarem associação com o grau histológico, ao se aplicar oscore (somatório dos achados dos critérios na RNM) encontramos, que a cada ponto a mais aumentase a chance de tumor de alto grau em 2 vezes (OR 2,0; p 0,014). CONCLUSÃO: A utilização da RNM como ferramenta para predizer o grau histológico dos sarcomas de partes moles se demonstrou eficiente. Os critérios relacionados ao padrão de crescimento infiltrativo tumoral identificados na RNM como sinal de alta intensidade peritumoral em T2 e realce pós-contraste peritumoral foram positivos para predizer alto grau histológico.

INTRODUCTION: Sarcomas are rare malignant tumors and comprise a heterogeneous group of tumors, with more than 60 histological subtypes. The importance of classifying the histological grade is fundamental for defining the line of treatment to be followed. The search for methods that increase the accuracy in the definition of high degree must be constant. OBJECTIVE: To evaluate the role of Magnetic Resonance Imaging in predicting the histological grade in soft tissue sarcoma. MATERIALS AND METHODS: This is an observational, cross-sectional retrospective study. Inclusion criteria were patients over 18 years old who underwent guided core needle biopsy or external pathology review, MRI exam, and primary tumor surgery at the ACCCC from January 2015 to June 2022. Patients who underwent surgical biopsy were excluded. The main variables were tumor size, age, location, histological subtype, histological grade of the biopsy and of the surgical specimen­ using a classification of high grade as G2 and G3 of the FNCLCC and low grade as G1. MRI radiological criteria (size, margin definition (regular or irregular), heterogeneity of the T2 signal (greater than 50% or less than 50%), high intensity peritumoral signal on T2 (peritumoral edema present or absent), post- peritumoral contrast (present or absent) were evaluated by two radiologists from our institution. In addition, a score was calculated to evaluate the prediction of histological grade, through the sum of the positive criteria analyzed (considering it to be 0 (zero), when no criterion was identified to 5 (five) when all criteria are positive). We used the Chi-square association test between the MRI items and the histological grade of the surgical specimen, and then we used the logistic regression test to identify the independent association. P values < 0.05 were considered significant. RESULTS: After applying the inclusion and exclusion criteria, sixty-eight patients wereincluded. Thirty women and 38 men. The biopsy identified 54 highgrade tumors and 14 low-grade tumors. In the surgical specimen, we had 52 high-grade and 16 low-grade tumors. The most common type of biopsy was ultrasound-guided core needle biopsy with 54.4% of cases. Liposarcoma with 17 (25%) cases were most frequently, followed by synovial sarcoma 17.6% and leiomyosarcoma 10.3%, as well as location in the extremity with 64.7%. We identified most tumors larger than 5cm, corresponding to 89.7%. The regularity of the margins 43 (63.2%) were regular, and only 25 (36.8%) considered irregular. In the evaluation of tumor heterogeneity in T2, there were 36 (52.9%) against 32 (47.1%). Peritumoral high-intensity T2-weighted signal was present in 45 (66.2%), whereas peritumoral post-contrast enhancement was present in 31 (45.6%). The score was: two with score 0 (zero), 10 with score 1 (one), 16 with score 2 (two), 13 with score 3 (three), 18 with score 4 (four) and nine with score 5 (five). The MRI criteria that demonstrated an association with histological grade were peritumoral high-intensity T2-weighted signal (p<0.001) and peritumoral post-contrast enhancement (p=0.006). In logistic regression, the presence of peritumoral high-intensity T2-weighted signal (OR 11.8) and peritumoral post-contrast enhancement (OR 8.8) were also predictive of high-risk tumor. Although the other MRI criteria are not associated with the histological grade, when applying the score (sum of the MRI findings of the criteria), we found that each additional point increases the chance of a high-grade tumor by 2 times (OR 2.0; p 0.014). CONCLUSION: The use of MRI as a tool to predict the histological grade of soft tissue sarcomas proved to be efficient. The criteria related to the infiltrative tumor growth pattern identified on MRI as peritumoral high-intensity T2-weighted signal and peritumoral post-contrast enhancement were positive for predicting histological high grade.

Avaliação clínica e epidemiológica de sarcomas: estudo retrospectivo de base de registro hospitalar de câncer do Estado de São Paulo / Clinical and epidemiological evaluation of Sarcomas: a retrospective study based on the hospital cancer registry in the State of São Paulo

Introdução: Os sarcomas são tumores raros e heterogêneos com origem mesenquimal. Atualmente são reconhecidos mais de 80 subtipos histológicos distintos. O tratamento compreende cirurgia, radioterapia e quimioterapia. A taxa de recidiva tumoral após a cirurgia e a sobrevida variam de acordo com o subtipo, graus histológicos e o estádio TNM. Por ser uma neoplasia rara, há inúmeras barreiras para o adequado diagnóstico e tratamento dos sarcomas.Objetivos: avaliar as características sociodemográficas e clínicas de pacientes com sarcomas tratados no estado de São Paulo. Objetivos secundários: Avaliar a frequência de sarcomas de partes moles no estado de São Paulo, as características sociodemográficas e clínicas, avaliar os padrões de tratamento, analisar a sobrevida global dos pacientes, analisar possíveis fatores prognósticos relacionados com a sobrevida. Metodologia: estudo observacional, de coorte retrospectiva, descritivo, de pacientes com diagnóstico de Sarcomas de Partes moles identificados na rede de registros hospitalares de câncer do Estado de São Paulo, Brasil, no período de 2008 a 2018. Foram analisadas variáveis sociodemográficas (sexo, idade, grau de escolaridade, tipo de instituição de tratamento e sistema de saúde), tempo do diagnóstico até o início do tratamento, subtipo histológico, localização do tumor primário, estadiamento TNM e tipo de tratamento empregado (cirurgia, radioterapia, quimioterapia e combinações). Foi realizada uma análise descritiva das variáveis, descrevendo as distribuições de frequência absoluta (n) e relativa (%) para as variáveis qualitativas, e as principais medidas resumo, como a média, desvio padrão, mediana, valores mínimo e máximo para as variáveis quantitativas. Para avaliar a associação entre variáveis qualitativas, foi utilizado o teste qui-quadrado ou o teste exato de Fisher, quando apropriado. A sobrevida global foi estimada pelo método de Kaplan Meier e a comparação entre as curvas de sobrevida foi feita pelo teste de log-rank. O modelo de regressão de Cox foi empregado para identificar variáveis relacionadas com o prognóstico. Adotou-se valor de significância de p de 0,05. Resultados foram incluídos na análise 3626 pacientes. A idade média foi de 51 anos (18-99 anos). Houve um discreto predomínio do sexo feminino (50,2%). A minoria tinha nível de ensino superior (10%), o tratamento ocorreu no SUS em 63,4% e na capital em 54,7% e 58,2% iniciaram o tratamento em menos de 60 dias. Houve predomínio de lipossarcoma e leiomiossarcoma (17,9% e 15,0%, respectivamente) e 45,8% tinham tumor nas extremidades e 31,5% no retroperitôneo. De acordo com o TNM, houve 6,4% estádio IA, 11,5% IB, 3,9% IIA, 6,1% IIB, 22% III, 20,8% IV, 11,3% X e 18,0% Y. Cirurgia, radioterapia e quimioterapia foram empregadas em 70,1%, 32,2% e 43,9%, respectivamente. Os pacientes tratados no SUS tiveram maior tempo para início do tratamento (76 x 58 dias, p <0,001) e foram tratados com menos cirurgia e radioterapia (68,7%x77,2%, p <0,001 e 31,8% x 37,2%, p 0,012, respectivamente). A sobrevida global mediana foi de 49 meses e a sobrevida global em 1, 2 e 5 anos foi de 76%, 63% e 46%, respectivamente. Na análise multivariada de Cox idade, local de tratamento, nível de escolaridade e sexo foram variáveis independentes para sobrevida global. Conclusão: Identificamos que houve atraso no início do tratamento após o diagnóstico para uma grande parcela dos pacientes. Além disso, encontramos discrepância significativa no tempo para início do tratamento de acordo com o sistema atendimento. Os pacientes do sistema público de saúde tiveram maior tempo para iniciar o tratamento. Nossos achados apontam para a existência de disparidades no sistema de saúde e reforçam a necessidade de mais estudos para entender melhor as barreiras enfrentadas pelos pacientes com diagnóstico de sarcomas.

Introduction: Sarcomas are rare and heterogeneous tumors with mesenchymal origin. Treatment includes surgery, radiotherapy, and chemotherapy. The tumor recurrence rate after surgery and survival varies according to the histological subtype. More than 80 distinct histological subtypes are currently recognized. Because it is a rare neoplasm, there are numerous barriers to proper diagnosis and treatment. Objectives: evaluation of sociodemographic and clinical characteristics of patients with sarcomas treated in the state of São Paulo. Secondary objectives: To evaluate the frequency of soft tissue sarcomas in the state of São Paulo, socio-demographic and clinical characteristics, evaluate treatment standards, analyze the overall survival of patients, and analyze possible prognostic factors related to survival. Methodology: observational, cohort, descriptive, retrospective study of patients diagnosed with soft tissue sarcomas identified in the network of hospital cancer registries in the state of São Paulo, Brazil, from 2008 to 2018. Socio demographic variables were analyzed (gender, age, educational level, type of treatment institution, health system), time from diagnosis to start of treatment, histological subtype, primary tumor location, tumor size, TNM staging, type of treatment employed (surgery, radiotherapy, chemotherapy and combinations). Statistical analysis: a descriptive analysis of the variables was performed, describing the absolute (n) and relative (%) frequency distributions for the qualitative variables, and the main summary measures, such as mean, standard deviation, median, minimum and maximum values were calculated. To assess the association between qualitative variables, the chi-square test or Fisher's exact test were used when appropriate. Overall survival was estimated using the Kaplan-Meier method and a comparison between survival curves was performed using the logrank test. A significance value of p value of 0.05 was adopted. Results: A total of 3626 patients were included in the analysis. The median age was 51 years (range 18 to 99 years). There was a slight predominance of females (50.2%). The minority had a higher education level (10%); the treatment took place in the public health care system (SUS) in 63.4% and in the capital in 54.7%, and 58.2% started treatment in less than 60 days. There was a predominance of liposarcoma and leiomyosarcoma (17,9.4% and 15.0%, respectively) and 45.8% had tumor in the extremities and 31.5% in the retroperitoneum. According to the TNM, there were 6.4% stage IA, 11.5% IB, 3.9% IIA, 6.1% IIB, 22% III, 20.8% IV, 11.3% X and 18.0% Y. Surgery, radiotherapy and chemotherapy were used in 70.1%, 32.2% and 43.9%, respectively. Patients treated at SUS had a longer time to start treatment (76 x 58 days, p <0.001) and were treated with less surgery and radiotherapy (68.7% x 77.2%, p <0.001 and 31.8% x 37.2%, p 0.012, respectively). The median overall survival was 49 months and the overall survival at 1, 2 and 5 years was 76%, 63% and 46%, respectively. In Cox's multivariate analysis, age, sex, educational level and local of treatment were independent variables for overall survival. Conclusion: We identified that there was a delay in starting treatment after diagnosis for a large proportion of patients. Furthermore, we found a significant discrepancy in the time to start treatment according to the care system. Patients in the public health system had longer time to start treatment. Our findings point to the existence of disparities in the healthcare system and reinforce the need for more studies to better understand the barriers faced by patients diagnosed with sarcomas.