Fibrocartilaginous mesenchymoma: a clinicopathological analysis of four cases / 中华病理学杂志

Objective: To investigate the clinical, radiological, histological and molecular features and the differential diagnosis of fibrocartilaginous mesenchymoma (FM). Methods: Four cases of FM diagnosed in the Department of Pathology, the Sixth People's Hospital Affiliated to Shanghai Jiaotong University School of Medicine from 2020 to 2022 were analyzed. Related literature was also reviewed. Results: Case 1 was a 10-year-old girl with bone destruction in the sacrum and L5 articular processes revealed by CT scan. Case 2 was a 7-year-old girl with an aggressive lesion in her right distal ulna. Case 3 was an 11-year-old boy with a lesion in the metaphysis of his left proximal tibia. Case 4 was an 11-year-old boy with bone destruction in the distal portion of a radius. Microscopically, the four tumors all consisted of numerous spindle cells, hyaline cartilage nodules, and bone trabeculae. The hypocellular to moderately cellular spindle cell component contained elongated cells with slightly hyperchromatic, mildly atypical nuclei arranged in bundles or intersecting fascicles. Benign-appearing cartilaginous nodules of various sizes and shapes were scattered throughout the tumors. There were areas mimicking epiphyseal growth-plate characterized by chondrocytes arranged in parallel columns and areas of enchondral ossification. The stroma was rich in mucus in case 1. Mutation of GNAS and IDH1/IDH2 and amplification of MDM2 gene were not found in any of the three tested cases. Conclusions: FM is very rare and tends to affect young patients. It most frequently occurs in the metaphysis of long tubular bones, followed by the iliac-pubic bones and vertebrae. FM is characterized by a mixed population of spindle cells, hyaline cartilage nodules and trabeculae of bone, without specific immunophenotypes and molecular alternations. As a borderline, locally aggressive neoplasm, surgical removal with a wide margin is generally the treatment of choice for FM.

Angiomixoma agresivo postrasplante renal / Aggresive angiomyxoma after renal transplantation

La condición de inmunosuprimido aumenta el riesgo de cáncer en trasplantados renales, en comparación a la población general. La mejor supervivencia de esta población en los últimos años ha convertido a las neoplasias y a la enfermedad cardiovascular en las principales causas de morbi-mortalidad. Presentamos el caso de un paciente trasplantado renal que desarrolló cuatro años después del trasplante una forma inusual de tumor mesenquimatoso, el angiomixoma agresivo, que requirió resección quirúrgica amplia.

The condition of immunosuppressed increases the risk of cancer in kidney transplant patients, as compared to the general population. The best survival of inmunosupressed patients in recent years has turned both neoplasms and cardiovascular diseases into the main causes of morbidity and mortality. We present the case of a renal transplanted patient who developed an unusual form of mesenchymal tumor such as the aggressive angiomyxoma, four years after the implant and requiring wide surgical resection.

Endoscopic Ultrasonography in the Diagnosis and Treatment Strategy Choice of Esophageal Leiomyoma

OBJECTIVES: Esophageal leiomyoma is the most common benign tumor of the esophagus, and it originates from mesenchymal tissue. This study analyzed the clinicopathological characteristics of esophageal leiomyoma and aimed to evaluate the role of endoscopic ultrasonography in the diagnosis and treatment selection for these lesions. METHODS: Two hundred and twenty-five patients who had suspected esophageal leiomyomas in endoscopic ultrasonography were enrolled at the Endoscopy Center of The First Affiliated Hospital, Zhejiang University from January 1st, 2009 to May 31th, 2015. The main outcomes included the demographic and morphological characteristics, symptoms, comparisons of diagnosis and treatment methods, adverse events, and prognosis. RESULTS: One hundred and sixty-seven patients were diagnosed as having an esophageal leiomyoma by pathological examination. The mean patient age was 50.57±9.983 years. In total, 62.9% of the lesions originated from the muscularis mucosa, and the others originated from the muscularis propria. The median distance to the incisors was 30±12 cm. The median diameter was 0.72±0.99 cm. As determined by endoscopic ultrasonography, most existing leiomyomas were homogeneous, endophytic, and spherical. The leiomyomas from the muscularis mucosa were smaller than those from the muscularis propria and much closer to the incisors (p<0.05). SMA (smooth muscle antibody) (97.2%) and desmin (94.5%) were positive in the majority of patients. In terms of treatments, patients preferred endoscopic therapies, which led to less adverse events (e.g., intraoperative bleeding, local infection, pleural effusion) than surgical operations (p<0.05). The superficial leiomyomas presented less adverse events and better recovery (p<0.05) than deep leiomyomas. CONCLUSION: Endoscopic ultrasonography has demonstrated high accuracy in the diagnosis of esophageal leiomyomas and provides great support in selecting treatments; however, EUS cannot completely avoid misdiagnosis, so combining it with other examinations may be a good strategy to solve this problem.

Primary osteosarcoma of the breast: pathological and imaging findings / Osteossarcoma primário da mama: achados patológicos e de imagem

SUMMARY Primary osteosarcoma of the breast (POB) is an extremely rare and aggressive tumor. Differential diagnosis of POB includes osteosarcoma of the chest wall and metaplastic breast carcinoma. Imaging tests that exclude the existence of a direct connection between the tumor and chest wall, as well as histopathological and immunohistochemical studies that rule out the presence of an epithelial component are required for the diagnosis of POB. We report a case of a 69-year old woman with POB. Imaging and pathological findings are presented. Therapeutic approach is discussed in the light of current knowledge, including potential complications.

RESUMO O osteossarcoma primário da mama (OPM) é um tumor extremamente raro e agressivo. O diagnóstico diferencial do OPM inclui o osteossarcoma da parede torácica e o carcinoma metaplásico da mama. Exames de imagem que excluam a existência de uma conexão direta entre o tumor e a parede torácica, e estudos histopatológico e imuno-histoquímico que descartem a presença de um componente epitelial são necessários para o diagnóstico de OPM. Relatamos um caso de OPM em uma mulher de 69 anos de idade. Os achados de imagem e patológicos são apresentados. A abordagem terapêutica é discutida à luz do conhecimento atual, incluindo potenciais complicações.

Mesenquimoma fibrocartilaginoso de húmero proximal: reporte de un caso / Fibrocartilaginous mesenchymoma of the proximal humerus: case report

El mesenquimoma fibrocartilaginoso (MFC) es un tumor raro, que afecta principalmente a los huesos largos. Se han reportado pocos casos desde su descripción. Presentamos un caso de un niño de 4 años de edad con MFC situado en el húmero. Las radiografías mostraron una lesión lítica expansiva situada en la región metafisaria del húmero proximal. La resonancia magnética objetivó expansión del tumor a partes blandas. La anatomía patológica fue confirmatoria de MFC. El paciente fue tratado con curetaje, fenolización adyuvante y sustituto óseo mezclado con aspirado de médula ósea. A los dos años de seguimiento, no se evidenció recidiva. El MFC debe ser tenido en cuenta entre los diagnósticos diferenciales en lesiones óseas líticas en niños y adolescentes.

Fibrocartilaginous mesenchymoma (FCM) is a rare tumor that primarily affects the long bones. Few cases have been reported since its description. A case of a 4-year-old boy with FCM located in the humerus is presented. Radiological examination showed an expansive lytic lesion located in the metaphyseal proximal humerus. Magnetic resonance imaging showed soft tissue expansion. Histopathological diagnosis was confirmatory of FCM. Curettage, adjuvant phenolization, and bone grafting with bone substitute, and autologous bone marrow was performed. During a follow-up period of 2 years, there was no evidence of disease progression. FCM should be considered in the differential diagnosis of lytic bone lesions in children and adolescents.

Tumores mesenquimales diagnosticados en la unidad de gastroenterología del HMPC entre los años 2007 aL 2009 / Mesenchymal tumors diagnosed in the gastroenterology unit of HPMC from 2007 to 2009

Se evaluaron las historias médicas con diagnóstico de tumores mesenquimales confirmados por análisis inmunohistoquímico entre los años 2007 y 2009. El objetivo fue describir a la población según características clínicas, epidemiológicas, diagnósticos por imágenes y análisis anatomopatológico. Se obtuvieron 11 pacientes con diagnósticos de GIST(7), Leiomiomas(2), GANT(1) y Leiomiosarcoma(1). Del género femenino (82%) y masculino (18%). Con un promedio de 55 años de edad. Clínicamente presentaron dolor abdominal (45%), mareos (27%), pirosis (9%) y disfagia (1%). El 18% permaneció asintomático. Entre los signos encontrados figuran: melena (36%), pérdida de peso (27%), palidez cutánea (9%) y vómitos (9%). El 36% de los pacientes no presentaron hallazgos al examen físico. Se realizó ultrasonido abdominal en el 100% de los pacientes, con hallazgos patológicos relacionados con el tumor en el 27 % de los casos. Al 90% de los pacientes se les realizó una Endoscopia Digestiva Superior, el 82% de los hallazgos se describieron como Tumores Submucosos y 9% se reportó como normal. Las lesiones se ubicaron en el estómago (60%), 27% en el intestino delgado ( duodeno y yeyuno); 9% en esófago y 9% en retroperitoneo. El ultrasonido endoscópico fue practicado al 82% de los pacientes, con un porcentaje de aciertos diagnósticos en el 90% de los tumores localizados en esófago, estómago e intestino delgado, y de 87,5% si se incluye el tumor retroperitoneal. La Tomografía Computada (TC) de abdomen fue practicada en 45% de los pacientes, con hallazgos patológicos en el 100% de los casos, descritos como Lesiones Ocupantes de Espacio (LOE), en estómago, intestino, esófago y retroperitoneo; y en el 20% de los pacientes se encontró enfermedad metastásica hepática Recibieron tratamiento quirúrgico el 82%, el 18% restante no se realizó por contraindicación médica o se encuentran en espera del procedimiento. Y un 18% de los pacientes recibieron tratamiento médico con Imatinib...

We evaluated the clinical records with a diagnosis of mesenchymal tumors confirmed by immunohistochemical analysis, from 2007 to 2009. The objective was to describe the population according to clinical and epidemiological features, diagnostic imaging and histopathological analysis. We found 11 patients with diagnoses of GIST(7), leiomyoma(2), leiomyosarcoma(1) and GANT(1); (82%) female and (18%) male, with a mean age of 55 years. They clinically presented abdominal pain (45%), dizziness (27%), heartburn (9%) and dysphagia (1%). 18% remained asymptomatic. Among the symptoms were: melena (36%), weight loss (27%), paleness (9%) and vomiting (9%). 36% of the patients had no findings at physical examination. Abdominal ultrasound was performed in 100% of the patients, with pathological findings related to the tumor in 27% of cases. In 90% of patients an upper digestive endoscopy was performed. 82% of findings were described as submucosal tumors and 9% was reported as normal. Lesions were located in stomach (60%); 27% in small bowel (duodenum and jejunum), 9% in esophagus and 9% in retroperitoneum. Endoscopic ultrasound was performed to 82% of patients, with a diagnostic accuracy of 90% for the tumors located in esophagus, stomach and small bowel; and 87,5% if the retroperitoneal tumor is included. The abdomen Computed Tomography (CT) was performed in 45% of patients with pathological findings in 100% of the cases, described as Space occupying lesion (SOL) in stomach, intestine, esophagus and retroperitoneum; and, in 20% of patients metastatic liver cancer was found. 82% received surgical treatment, the remaining 18% was not performed due to contraindication or are waiting for the procedure. And 18% of patients received medical therapy with Imatinib...

GIST de colon: diagnóstico y factores pronósticos / Colon GIST: diagnosis and prognostic factors

Los tumores gastrointestinales derivados del mesénquima se han denominado en general leiomiomas o schwan nomas por sus semejanzas histológicas con el tejido muscular y neural, pudiendo ser en su evolución benignos o malignos. En el último tiempo se ha informado un creciente número de tumores derivados del mesénquima cuyo origen histológico es difícil de definir y son denominados GIST. Su diagnóstico se basa fundamentalmente en técnicas inmunohistoquímicas. Localizados preferentemente en estómago e intestino delgado, su aparición en colon es excepcional. Su potencial maligno y pronóstico se basa fundamentalmente en el tamaño, localización y grado histológico

Stromal sarcoma of the breast: a case report.

Malignant mesenchymal tumours of the breast are rare neoplasms. Their incidence varies from 1%-3% of all malignant breast tumours (1). Stromal sarcoma of the breast is an extremely uncommon tumour (2). We present a rare case of advanced stromal sarcoma of the breast successfully managed with radical surgery and radiotherapy.

Ectomesenquimoma maligno; informe de un caso / Malignant ectomesen chimoma: report of a case

Se informan los hallazgos clínicos y patológicos de un ectomesenquimoma maligno de partes blandas. Los ectomesenquimomas malignos son tumores compuestos por derivados de la cresta neural y uno o más elementos mesenquimatosos malignos, por lo general rabdomiosarcoma, condrosarcoma, angiosarcoma y liposarcoma maligno y liposarcoma. En esta neoplasia, el tratamiento más adecuado es el quirúrgico, pues la quimioterapia y la radioterapia no parecen modifiar el curso de la enfermedad. En este caso el tratamiento fue quirúrgico, sin datos de actividad tumoral a los 16 meses subsecuentes a la operación.

Bone tumors of mixed origin: osteoliposarcoma and osteo-rhabdomyosarcoma

One: A complete perusal of the literature velealed twenty cases of primary liposarcoma of bone acceptable as such to the authors. These were tabulated as to location and age. 2. Eight cases of osteo-liposarcoma, primary in bone, were encountered in the literature and an additional case was reported by the authors. 3. The authors described for the first time in the literature a new primary tumor of bone of mixed origin: osteo-rhabdomyosarcoma. After careful perusal of the literature they added three additional casesÑ two cases56, 58 previously reported as primary rhabdomyosarcoma of bone, which on carreful evaluation of the radiographs in said publications and the paucity of microphotographs they considered to be osteo-rhabdomyosarcomas, and the other case, previously reported as malignant mesenchymoma of the sternum following radiotherapy for breast cancer. 4. The authors prefer to classify these tumors (osteo-liposarcoma and osteo-rhabdomyosarcoma) as "Tumors of Mixed Origin" and not as "Malignant Mesenchymomas". 5. A complete review of the literature revealed 219 reported "with additional mesenchymal component", among which only nine (9) contained a bonafide rhabdomyosarcomatous component. The rest exhibited other mesenchymal tumors as osteogenic sarcoma, fibrosarcoma, malignant fibrous histiocytomas, angiosarcoma, and undifferentiated sarcoma. The authors recommend to continue classifying these tumors as chondrosarcomas with additional mesenchymal component or even as ...

Mesenquimoma maligno (osteo-liposarcoma) primario de hueso. Reporte de un caso y revisión de la literatura / Mesenchymoma (osteo-liposarcoma) bone primary. Report of a case and a review of the literature

Se presenta un caso de mesenquimoma maligno (osteo-liposarcoma) primario de hueso, localizado en la tibia derecha de una paciente de 15 años de edad. Esta es una neoplasia maligna en donde histológicamente se pueden reconocer áreas de osteosarcoma entremezcladas con áreas de liposarcoma. El estudio radiológico mostró zonas irregulares de osteolisis en la metáfisis superior y en la epífisis de la tibia derecha, las cuales alternaban con zonas densas de límites imprecisos. El pronóstico parece ser igual al del osteosarcoma. El autor cree que éste es el séptimo caso reportado según la literatura revisada

Mesenquimoma maligno con recidiva tardía como liposarcoma: presentación de un caso y revisión de la literatura / Malignant mesenchymoma with late recurrence as liposarcoma: report of a case and literature review

El mesenquimoma maligno es un tumor de tejidos blandos de origen mesenquimatoso, compuesto por células de dos o más estirpes no relacionadas; sus localizaciones más frecuentes son tejidos profundo de las extremidades inferiores, retroperitoneo, tórax y tejidos blandos de otras partes del cuerpo. Se presenta el caso de un paciente con un mesenquimoma retroperitoneal de gran tamaño (20 x 25 cm) tratado con resección amplia. El estudio histopatológico informó componentes de liposarcoma, osteosarcoma y condrosacorma. El tumor reapareció casi ocho años después, y se extirpó una tumoración de 10 x 12 cm con patrón histopatológico de liposarcoma. El enfermo está asintomático 24 meses después. El caso informado es de interés por tratarse de un mesenquimoma que recurre en una sola línea de diferenciación, comportamiento poco común de estos tumores, además de la buena evolución hasta la fecha de publicación sin terapéutica adyuvante