Tumor Desmoplásico Intraabdominal de células redondas y pequeñas. Abordaje médico quirúrgico y fisioterapéutico. A propósito de un caso / On the subject of a case: Intra-abdominal desmoplastic small and round cell tumor: a medical-surgical and physiotherapeutic approach

El Tumor Desmoplásico Intraabdominal de células pequeñas y redondas es un tumor agresivo, poco frecuente, el pronóstico es pobre y presenta una supervivencia total de 15% a los 5 años. Se presenta el caso clínico de un paciente con diagnóstico de Tumor Desmoplásico, sometido a cirugía citoreductora y quimioterapia intraperitoneal hipertérmica internado durante 46 días en el Hospital de Clínicas de la Facultad de Ciencias Médicas, donde recibió un tratamiento multidisciplinario. El tratamiento kinésico inició posterior a la cirugía y continuó de manera ambulatoria logrando finalmente la marcha independiente, completándose un total de 20 sesiones. La evolución clínica kinésica quirúrgica demostró la importancia del trabajo interdisciplinario desde el primer contacto con el paciente. A pesar del desenlace desfavorable del paciente, en el tiempo de recuperación post quirúrgico se pudo acceder a las terapéuticas logrando importantes resultados respecto a la independencia física. El reporte presentado corresponde al tercer caso de esta patología, sometido a cirugía en el Hospital de Clínicas de la Facultad de Ciencias Médicas, el aporte de este informe nos permite valorar el manejo integral de un paciente oncológico.

The desmoplastic small round cell tumor is a rare aggressive tumor, the prognosis is poor and it has a total survival of 15% at 5 years. We presented the clinical case of a patient with a diagnosis of desmoplastic tumor, who underwent cytoreductive surgery and hyperthermic intraperitoneal chemotherapy, hospitalized for 46 days, where he received multidisciplinary treatment.Physical Therapy treatment began after surgery and continued on an outpatient basis, finally achieving independent walking, completing a total of 20 sessions. The clinical evolution demonstrated the importance of interdisciplinary work from the first contact with the patient. Despite the unfavorable outcome of the patient, in the post-surgical recovery time, therapeutics could be accessed, achieving important results regarding physical independence. The report presented corresponds to the third case of this pathology, submitted to surgery at the Hospital de Clínicas of the Faculty of Medical Sciences, the contribution of this report allows us to assess the comprehensive management of an oncological patient.

Tumor desmoplásico de célula pequeña y redonda paratesticular/testicular: reporte de caso y revisión de la literatura / Paratesticular/testicular desmoplastic round small cell malignant tumor: case report and literature review

Resumen El tumor desmoplásico de célula redonda y pequeña (TDCRP) es una patología neoplásica maligna agresiva y poco común. Afecta predominantemente a hombres entre la segunda y tercera década de la vida. Los pacientes que la padecen tienen un pronóstico pobre, con una supervivencia global a 5 años de hasta el 30%. Por lo general se presenta como una masa en la cavidad abdominal, frecuentemente multifocal. Para su tratamiento se recomienda un enfoque multimodal con cirugía, quimioterapia y radioterapia. Poco más de 20 casos de TDCRP a nivel testicular/paratesticular se han reportado en la literatura. A continuación, se presenta un caso ilustrativo en esta localización, se discute el caso y se realiza revisión de la literatura.

Abstract Desmoplastic small round cell tumor (DSRCT) is an aggressive and rare malignant neoplasm. It mainly affects young men in their twenties and thirties. Patients with it have a poor prognosis, with a 5-year survival rate of up to 30%. It generally presents as a mass in the abdominal cavity, often multifocal. A multimodal approach is recommended for its treatment, with surgery, chemotherapy, and radiotherapy. Just over 20 cases of testicular/paratesticular DSRCT have been reported in the literature. Below, we present an illustrative case in this location, we discuss the case and review the literature.

Desmoplastic small round cell tumor of the ovary: A case report with a new modality of treatment and review of the literature

Abstract Objective Desmoplastic small round cell tumor (DSRCT) is a rare intraabdominal neoplasm that grows along serosal surfaces and is primarily found in young men. To Keywords date, only 16 cases of ovarian DSRCT have been previously reported in women in the English literature, and no large population-based studies on this topic exist. Case Report We report the case of a 19-year-old virgo with unremarkable past medical history, initially presented with abdominal fullness. After being treated with the optimal treatment modality (primary and secondary surgical debulking, unique chemotherapy, protocol and adjuvant radiotherapy), the patient has remained without tumor disease for 40 months. Conclusion Although the best therapy for patients with DSRCT has yet to be determined, combining complete surgical resection, adjuvant chemotherapy, and radiotherapy is required to prolong survival and to achieve proper quality of life.

Tumor desmoplásico de células pequeñas y redondas. Presentación de caso y revisión de la literatura / Desmoplastic Small Round Cell Tumor. A Case Report and Literature Review

El tumor desmoplásico de células pequeñas y redondas (TDCPR) es una neoplasia maligna rara, con curso clínico agresivo y mortalidad elevada. Se presenta el caso de un hombre de 21 años de edad, quien consultó por dolor abdominal de intensidad moderada, irradiado al flanco derecho, fiebre y pérdida de peso. En tomografía abdominal con medio de contraste se documentó una gran masa intraperitoneal con áreas de necrosis central y extensión a la pelvis, además de lesiones hepáticas de aspecto neoplásico secundario. El diagnóstico se confirmó mediante biopsia percutánea guiada por ultrasonido, que mostró extensa infiltración por tumor maligno, constituido por células con núcleos vesiculosos de cromatina clara, citoplasma eosinófilo e inmunohistoquímica compatible con dicho tumor. En este artículo se hace una confrontación del caso con los hallazgos descritos en otras series publicadas en la literatura y una revisión clínica del tema.

Desmoplastic small round cell tumor (DSRCT) is a rare malignant neoplasm with an aggressive clinical course and high mortality. The case of a 21-year-old man is presented, who consulted for abdominal pain of moderate intensity radiating to the right flank, fever and weight loss. Contrast abdominal tomography was performed, documenting a large intraperitoneal mass with areas of central necrosis and extension to the pelvis, in addition to secondary neoplastic liver lesions. The diagnosis was confirmed by ultrasound-guided percutaneous biopsy, which reported extensive infiltration by malignant tumor, consisting of cells with vesicular nuclei of clear chromatin, eosinophilic cytoplasm and immunohistochemistry compatible with said tumor. This case report is compared with the findings described in other series published in the literature and a clinical review of the subject is made.

Infarto medular en paciente con aneurisma de aorta descendente con hematoma intramural / Medular Infarction in Patient with Descending Aorta Aneurysm with Intramural Hematoma

La isquemia medular (IM) es una emergencia médica causada por hipoperfusión o embolismo de la arteria espinal. El inicio de los síntomas es abrupto, y su presentación clínica y gravedad es amplia, dependiendo del segmento medular comprometido. Entre las causas más frecuentes están la enfermedad ateroesclerótica, las cirugías vasculares aórticas y, menos comúnmente, la disección aórtica (DA). La IM conlleva secuelas graves y alta mortalidad. Este artículo describe el caso de un paciente con infarto medular agudo como primera manifestación de un hematoma intramural (HIM) en un aneurisma de aorta torácica.

Spinal cord ischemia is a medical emergency due to hypoperfusion or embolization of the spinal artery. The onset of symptoms is abrupt and the clinical presentation depends on the medullary territory involved. The most frequent causes are atherosclerotic disease, aortic surgery and less commonly, aortic dissection, which is associated with serious complications and greatest risk of mortality. This article describes the case of a patient with an acute spinal infarction as the first manifestation of an intramural hematoma in a thoracic aortic aneurysm.

MRI Finding of Retroperitoneal Desmoplastic Small Round Cell Tumor with Hepatic Metastasis and Portal Vein Thrombosis: a Case Report

Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive malignancy common in young male patient. Typical imaging features of DSRCT include multiple soft tissue masses in the peritoneal cavity, omentum, or mesentery without an organ of origin. This report presents a rare manifestation of DSRCT revealing a solitary large retroperitoneal mass with hepatic metastasis and malignant portal vein thrombosis in 70-year-old women together with the review of literature. The tumor showed a hemorrhagic and necrotic mass with peripheral portion of T2 hypo-intensity and delayed enhancement that indicated desmoplastic stroma with dense cellularity.

Cirurgia Citorredutora e Quimioterapia Intraperitoneal Hipertérmica para Tratamento de Tumor Desmoplásico de Pequenas Células Redondas em Paciente Pediátrico: Relato de Caso / Cytoredutive Surgery and Chemohyperthemia for Desmoplasic Small Round Cell Tumor in Pediatric Patient: Case Report / Cirugia de Citoreducción y quimihipertermia el Tratamento de Tumor desmoplásico de Pequeñas Células Redondas en Paciente Pediátrico: Relato de Caso

Introdução: o tumor desmoplásico de pequenas células redondas (tdPcr) é uma neoplasia rara com comportamento clínico agressivo. trata-se do caso de um paciente com 7 anos de idade, sexo masculino, com tdPcr, matriculado no serviço de Pediatria do instituto nacional de câncer José alencar Gomes da silva. Relato do caso: Paciente iniciou o quadro com queixa de dor e aumento do abdome, ascite volumosa, febre e emagrecimento. nos exames de imagem, apresentava lesão hipodensa no segmento iV a do fígado, ascite volumosa, massa justa parietal no hemitórax direito. o laudo histopatológico foi compatível com tdPcr estádio iV. o paciente foi submetido à quimioterapia sistêmica com resposta completa nas lesões torácicas e redução importante da massa abdominal, restando lesões em cavidade pélvica. o paciente foi submetido à cirurgia com citorredução e hipertermoquimioterapia com cisplatina, e recebeu radioterapia abdominal adjuvante e quimioterapia. o paciente manteve-se estável, apresentando nova progressão e óbito 14 meses após a recidiva. Conclusão: a citorredução cirúrgica associada à hipertermoquimioterapia intraperitoneal permitiu a possibilidade de controle temporário da doença com boa qualidade de vida para o paciente.

Introduction: desmoplastic small round cell tumor (dsrct) is rare and highly aggressive mesenchymal tumor. objective: case report of a 7 y-o boy, diagnosticated with a dsrct, treated in the Pediatric service at national cancer institute José alencar Gomes da silva. Case report: He presented with abdominal pain, abdominal mass, ascites, fever and slimming. computer tomography showed a hypodense tumor on iV hepatic segment, volumous ascitis, tumoral mass in right hemithorax. He was submitted to needle biopsy with histopatologic result as dsrct staged as iV. The patient was submitted to sistemic chemotherapy with complete response on thoracic tumor e abdominal tumor reduction, with stable pelvic lesions. an cytoredutive surgery with cisplatin hyperthermic intraperionteal chemotherapy. He received abdominal radiotherapy and chemotherapy. He presented with tumor progression and death after 14 months. Conclusion:cytoredutive surgery and Hyperthermic intraperitoneal chemotherapy permitted a temporary disease control with a good quality of life

Introducción: el tumor desmoplásico de pequeñas células redondas (tdPcr) es una neoplasia rara con comportamento clínico agresivo. objetivo: relatar el caso de un paciente con 7 años de edad, sexo masculino con tdPcr, matriculado en el servicio de Pediatria en el instituto nacional del cancer José alencar Gomes da silva. Relato del caso: el paciente inició el cuadro con dolor y aumento del abdomen, ascite voluminosa, fiebre y adelgazamiento. en los exámenes de imagen presentaba lesión hipodensa en el segmento iV a del hígado, ascite voluminosa, masa justa parietal en hemitórax derecho. el histopatológico fue compatible con el tdPcr estadio iV. el paciente fue sometido a quimioterapia sistémica con respuesta completa en las lesiones toráxicas y reducción importante de la masa abdominal, restando lesiones en la cavidad pélvica. el paciente fue sometido a cirurgía con citorreducción e hipertermoquimioterapia con cisplatina. recibió radioterapia abdominal adjuvante y quimioterapia. el paciente se mantuvo estable, presentando nueva progresión y óbito 14 meses después de la recidiva. Conclusión: la citoreducción cirúrgica asociada a la hipertermoquimioterapia intraperitoneal permitió la posibilidad de control temporario de la enfermedad con buena calidad de vida para el paciente.

Tumor Desmoplásico de Pequenas Células Redondas: Relato de caso / Desmoplastic Small-Round-Cell Tumor: Case Report / Tumor Desmoplásico de Células Pequeñas y Redondas: Relato de Caso

Introdução: O tumor desmoplásico de pequenas células redondas é uma rara neoplasia que se inicia e se espalha pela superfície peritoneal. Foi descrito pela primeira vez em 1989 e, em 1991, houve seu reconhecimento como entidade clínica e patológica distintas. Relato do caso: Homem de 34 anos apresentou quadro de dor abdominal e perda de peso, evoluindo para obstrução intestinal dois meses após. A laparotomia demonstrou grande massa abdominopélvica irressecável. O laudo anatomopatológico associado à imuno-histoquímica evidenciou diagnóstico de tumor desmoplásico de pequenas células redondas. A tomografia computadorizada confirmou derrame pleural bilateral, implantes peritoneais e massas abdominais e pélvicas. Realizou-se quimioterapia com carbo/taxol com intervalo de 21 dias. Substituiu-se o esquema para VAC/IE com intervalo de 21 dias, com resposta parcial, porém ainda se mantendo um tumor irressecável. Houve piora progressiva da performance do paciente, com evolução ao óbito por obstrução intestinal no 15º mês de seguimento. Conclusão: O tumor desmoplásico de pequenas células redondas, em razão da sua raridade, continua sendo um desafio para o diagnóstico e o tratamento.

Introduction: The desmoplastic small round cell tumor is a rare neoplasm that starts and spreads through the peritoneal surface. It was first described in 1989 and in 1991 was recognized as a distinct clinical and pathological entity. Case report: A 34-year-old man presented with abdominal pain and weight loss, progressing to an intestinal obstruction after two months. Laparotomy showed an unresectable abdominopelvic mass. Anatomopathological an immunohistochemistry analysis showed a desmoplastic small-round-cell tumor. Computerized Tomography showed bilateral pleural effusion, peritoneal implants, along with masses in the abdominal and pelvic region. Chemotherapy with carbo/taxol was administered at intervals of 21-days. Later, the chemotherapy was changed to VAC/IE at a 21-day interval, with a partial response, but it was still an unresectable tumor. There was a worsening in patient performance, and he died of an abdominal obstruction on the 15º month of follow-up. Conclusion: Due to its rarity, the desmoplastic small-round-cell tumor, is still a diagnostic and treatment challenge.

Introducción: El tumor desmoplásico de células pequeñas y redondas es una neoplasia rara que comienza y se disemina a través de la superficie peritoneal. Fue descrito por primera vez en 1989 y en 1991 fue reconocido como una entidad clínica y patológica distintas. Relato del caso: Un hombre de 34 años presentó dolor abdominal y pérdida de peso, progresando a una obstrucción intestinal después de dos meses. La laparotomía mostró una masa abdominopélvica irresecable. El análisis anatomopatológico e inmunohistoquímico mostró un tumor desmoplásico de células pequeñas y redondas. La tomografía computarizada mostró derrame pleural bilateral, implantes peritoneales y masas en la región abdominal y pélvica. Se administró quimioterapia con carbo/taxol en un intervalo de 21 días. Más tarde, la quimioterapia cambió a VAC/IE con un intervalo de 21 días, con una respuesta parcial, pero seguía siendo un tumor irresecable. Hubo un empeoramiento en el estado del paciente, y murió de una obstrucción intestinal en el 15º mes de seguimiento. Conclusión: Debido a su rareza, tumor desmoplásico de células pequeñas y redondas, sigue siendo un desafío de diagnóstico y tratamiento.

Clinical Characteristics and Treatment Outcome of the Desmoplastic Small Round Cell Tumor / 임상소아혈액종양

BACKGROUND: Desmoplastic small round cell tumor (DSRCT) is an aggressive malignancy with a poor prognosis. DSRCT is a rare disease, and therefore a standard treatment regimen has not been established. In this study, we reviewed the clinical characteristics and treatment outcomes of pediatric DSRCT patients.METHODS: We retrospectively reviewed the medical records of 5 DSRCT patients (2 boys, 3 girls) that were diagnosed and treated with DSRCT at Seoul National University Children's Hospital from January 1999 to January 2015.RESULTS: The median age at diagnosis was 11 years 5months (range 4 years 10 months-17 years 2 months). The most frequent symptoms were abdominal pain (60%). The primary sites were gastrointestinal tract, bladder, and omentum, and the involved sites were the liver, gastrointestinal tract, bladder and bone. Three patients had multiple metastases at diagnosis. Two patients underwent upfront surgical excision of primary tumor, and the remaining 3 patients received neo-adjuvant chemotherapy after the diagnosis was confirmed by using needle biopsy. Combination chemotherapy was administered to all patients in addition to radiotherapy (median dose 45 Gy, range 17.5-54 Gy). Four patients showed disease progression or relapse, resulting in a 20% overall survival rate. At the time of analysis, one patient is alive. She had localized disease at the time of diagnosis and were treated with upfront surgery, chemotherapy, and high-dose chemotherapy with autologous stem cell transplantation and radiotherapy.CONCLUSION: Patients with DSRCT have a poor prognosis, even after multimodal treatment. Further studies are needed to determine the prognostic factors of DSRCT.

Analysis of the diagnosis and treatment of desmoplastic small round cell tumor / 中华肿瘤杂志

<p><b>OBJECTIVE</b>To explore the clinical diagnostic features and treatment of desmoplastic small round cell tumor (DSRCT), and to improve the understanding and management of this tumor.</p><p><b>METHODS</b>The clinicopathological data of nine patients treated in our hospital from October 2004 to June 2014 were retrospectively analyzed and a review of the literature was made. The clinical manifestations, pathological characteristics, diagnosis and differential diagnosis, treatment and prognosis of this tumor were summarized and analyzed.</p><p><b>RESULTS</b>Nine patients with DSRCT, 5 males and 4 females, with an average age of 21 years (range 8-56 years) were included in this study. Ultrasound examination revealed irregular low-density mass shadow in the abdominal cavity. CT examination found that 6 cases had abdominal and retroperitoneal multiple solid tumor nodules, uneven density, and visible low density fluid area. Postoperative pathological examination revealed that the tumor cells were small, mostly elliptic, gathered to form clear structure of nests with clear irregular boundaries. The central portion of large tumor nests often showed necrosis. Scattered fibroblasts and large amount of hyalinization of collagen fibers were seen in the interstitial tissue around the nests. Six patients received laparotomy surgery, however, all failed to resect the tumor completely. Three patients received postoperative chemotherapy, i. e. two cases had carboplatin and paclitaxel chemotherapy, and one case of chemotherapy regimen not specified. Two patients had radiation and chemotherapy (no concrete plan was available). Another case was lost to follow-up. Two of the three patients without surgery received chemotherapy with CAP (cyclophosphamide+adriamycin+carboplatin) and total rectal lesions, pelvic and inguinal lymph nodes, ilium metastases radiation therapy. Another one patient received EP regimen (DDP+VP16) which was then changed into a TP chemotherapy alone. Eight of the nine cases died shortly after surgery, and only one patient treated with chemotherapy alone was still alive after 11 months of follow-up.</p><p><b>CONCLUSIONS</b>Desmoplastic small round cell tumor is a very rare, special type of soft tissue tumor, with very poor prognosis. This tumor may be preliminarily diagnosed according to the imaging characteristics and detection of tumor markers, however, final diagnosis is made by pathology. Surgery is the priority of treatment, combined with complementary radiation and chemotherapy.</p>

Clinical Characteristics and Treatment Outcome of the Desmoplastic Small Round Cell Tumor / 임상소아혈액종양

BACKGROUND: Desmoplastic small round cell tumor (DSRCT) is an aggressive malignancy with a poor prognosis. DSRCT is a rare disease, and therefore a standard treatment regimen has not been established. In this study, we reviewed the clinical characteristics and treatment outcomes of pediatric DSRCT patients. METHODS: We retrospectively reviewed the medical records of 5 DSRCT patients (2 boys, 3 girls) that were diagnosed and treated with DSRCT at Seoul National University Children's Hospital from January 1999 to January 2015. RESULTS: The median age at diagnosis was 11 years 5months (range 4 years 10 months-17 years 2 months). The most frequent symptoms were abdominal pain (60%). The primary sites were gastrointestinal tract, bladder, and omentum, and the involved sites were the liver, gastrointestinal tract, bladder and bone. Three patients had multiple metastases at diagnosis. Two patients underwent upfront surgical excision of primary tumor, and the remaining 3 patients received neo-adjuvant chemotherapy after the diagnosis was confirmed by using needle biopsy. Combination chemotherapy was administered to all patients in addition to radiotherapy (median dose 45 Gy, range 17.5-54 Gy). Four patients showed disease progression or relapse, resulting in a 20% overall survival rate. At the time of analysis, one patient is alive. She had localized disease at the time of diagnosis and were treated with upfront surgery, chemotherapy, and high-dose chemotherapy with autologous stem cell transplantation and radiotherapy. CONCLUSION: Patients with DSRCT have a poor prognosis, even after multimodal treatment. Further studies are needed to determine the prognostic factors of DSRCT.

Incidence and Survival of Pediatric Soft Tissue Sarcomas: Comparison between Adults and Children / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: Pediatric-type sarcomas such as rhabdomyosarcoma (RMS), Ewing sarcoma (EWS), primitive neuroectodermal tumor (PNET), and desmoplastic small round-cell tumor (DSRCT) are rare in adults, with limited studies on their prognosis and optimal treatment strategies. We aimed to examine the outcome of children and adult patients with RMS, EWS, PNET, and DSRCT and relevant prognostic factors. MATERIALS AND METHODS: We retrospectively reviewed 220 pediatric-type sarcoma patients at a single institution between 1985 and 2011. Comparisons were made in order to examine differences in demographics, disease characteristics, and survival. Survival analyses were performed using the Kaplan-Meier method with log-rank tests and Cox proportional hazards models. RESULTS: A total of 220 consecutive patients were identified at our institute. Median age was 15.6 years (range, 0 to 81 years) and there were 108 children (49%) and 112 adult patients (51%). According to histological classification, 106 patients (48.2%) had RMS, 60 (27.3%) had EWS, 50 (22.7%) had PNET, and 4 (1.8%) had DSRCT. With a median follow-up period of 6.6 years, the estimated median overall survival (OS) of all patients was 75 months (95% confidence interval [CI], 27.2 to 122.8 months) and median event-free survival (EFS) for all patients was 11 months (95% CI, 8.8 to 13.2 months). No significant difference in OS and EFS was observed between adults and children. In multivariate analysis, distant metastasis (hazard ratio [HR], 1.617; 95% CI, 1.022 to 2.557; p=0.040) and no debulking surgery (HR, 1.443; 95% CI, 1.104 to 1.812; p=0.012) showed independent association with worse OS. CONCLUSION: Metastatic disease and no surgical treatment are poor prognostic factors for OS among pediatric-type sarcomas for both adults and children.

Intra-abdominal desmoplastic small round cell tumor in a 45-year-old man: a case report

Intra-abdominal desmoplastic small round cell tumor is a rare entity with a few reports worldwide. This tumor commonly occurs in children and adolescent and occurrence in adult age group is very rare. Here, we describe an adult male with symptoms of intestinal obstruction due to abdominal mass, located in splenic flexure. After resection, the diagnosis of intra-abdominal desmoplastic small round cell tumor was established by pathologic and immunohistochemical studies. We emphasize that albeit being rare, this tumor should be mentioned as one of the differential diagnoses of widespread intra-abdominal lesions in any age group. A brief review of epidemiology, clinical manifestations as well as pathological and molecular features is also included in the paper.

ThinPrep Cytological Findings of Desmoplastic Small Round Cell Tumor with Extensive Glandular Differentiation: A Case Study

Desmoplastic small round cell tumor (DSRCT) is a rare and highly aggressive neoplasm. The cytological diagnosis of this tumor has only been reported in a few cases. In most of these cases, the diagnosis was made using fine-needle aspiration cytology. Most DSRCTs resemble disseminated carcinomatoses in their clinical manifestation as well as cytomorphologically, even in young-adult patients. These authors report a case of using peritoneal-washing and pleural-effusion ThinPrep cytology to diagnose DSRCT, with extensive glandular differentiation and mucin vacuoles. We found that fibrillary stromal fragment, clinical setting, and adjunctive immunocytochemical staining were most helpful for avoiding misdiagnosis.

Desmoplastic Small Round Cell Tumor Achieved Complete Remission after High-Dose Chemotherapy with Aggressive Local Treatment Modalities / 대한내과학회지

A desmoplastic small-round-cell tumor (DSRCT) is a rare, aggressive neoplasm that develops mostly in the abdominal cavity in children and young adults. We present a case of a 19-year-old male with right upper quadrant discomfort for 3 months. On abdominal computerized tomography, multiple huge and demarcated masses were found in the liver, retroperitoneal lymph nodes, and peritoneal and retroperitoneal cavities. Fine needle aspiration biopsy of the hepatic mass was performed and DSRCT was diagnosed by hematoxylin and eosin staining and immunohistochemical analysis. He was treated initially with high-dose systemic chemotherapy (alternating schedules of cyclophosphamide, vincristine, doxorubicin, ifosfamide, and etoposide), underwent two debulking surgeries and pelvic irradiation between systemic chemotherapy schedules, and achieved complete remission after the 15 months of treatment duration. We report this case to emphasize the importance of aggressive local treatment modalities as well as high-dose systemic chemotherapy for treatment of DSRCT even with initially unresectable or extensively metastatic presentation.

8-year survivor of unresectable intrapelvic desmoplastic small round cell tumor treated with concurrent chemoradiotherapy

Desmoplastic small round cell tumor is a rare malignant tumor that occurs primarily in young males. Here, a case of small round cell tumor in an adult male successfully treated with a curative concurrent chemoradiotherapy is presented. A 58-year-old man had an intrapelvic tumor. Surgical resection was attempted, but the tumor was unresectable. Needle biopsy was performed and the diagnosis was suggested to be desmoplastic small round cell tumor. Concurrent chemoradiotherapy was performed, and a complete response was obtained. This patient has been alive for 8 years after treatment with no evidence of disease. Concurrent chemoradiotherapy appears to be a useful treatment choice for unresectable desmoplastic small round cell tumor

[Desmoplastic small round cell tumor]

Desmoplastic small round cell tumor is a recently recognized and rare clinicopathologic entity with distinctive morphologic and immuno histochemical features .Moreover, specific cytogenetics abnormalities and molecular characteristics have been described and confirmed its identity. It is often located at the abdominal cavity, but can exceptionally be located at the cerebral level or some soft tissue and the bones of the members. We report a case of Desmoplastic small round cell involving gluteal muscle and iliac bone at a 25-year-old girl. Our patient had been treated by chemotherapy based on ifosfamide, doxorubicine, etoposide and cis platine. The evolution was marked by the tumoral progress and the death of the patient 18 months after the discovery of the disease. The second case is a 27-year old man .He had a retro peritoneal desmoplastic small round cell tumor treated by surgery alone. The evolution was marked by metastasis and the patient died 10 months after diagnosis. The prognosis of these tumors remains unclear in spite of the mulitidisciplinary treatment. Hence the necessity of new lines of therapeutic research

Clinicopathologic Features and Clinical Outcomes of Desmoplastic Small Round Cell Tumors in Abdomen

Desmoplastic small round cell tumor (DSRCT) is a rare malignant tumor that occurs mainly in the abdominal cavity and has a tendency to occur in young males. But it occurs in other primary sites including the central nerve system, lung, kidney and soft tissue like bone. Major symptoms include abdominal palpable mass, abdominal pain and distention. Useful studies for diagnosis are radiographic study including computerized tomogram and percutaneous pathologic study. DSRCT is an aggressive malignant neoplasm and its prognosis is poor. The overall survival at three years is about 30%. This report describes 5 cases of DSRCT since 2002. The median age of patients was 35 years. All patients are men that presented abdominal pain and abdominal mass. 3 patients underwent surgical resection of tumor and 4 patients received chemotherapy. The survival time of 2 expired patients was 39 and 28 months, individually. And the follow-up period of survival patients is 23~31 months.

Desmoplastic small round cell tumor of the stomach mimicking a gastric cancer in a child / 대한외과학회지

Intra-abdominal desmoplastic small round cell tumor (DSRCT) is a highly malignant tumor of uncertain histogenesis. Here we report a case of DSRCT involving the stomach, initially misdiagnosed as gastric cancer. A 12-year-old boy presented with upper abdominal pain developed 1 month prior. On gastroscopy, a 7-cm mass was noted involving the esophago-gastric junction to the fundus, and positron emission tomography showed multiple hot uptakes suggesting distant metastasis. Gastroscopic biopsy showed poorly differentiated malignant cells. We diagnosed as stage IV gastric cancer and treated with 6 cycles of chemotherapy. Laparotomy revealed a huge gastric mass along with peritoneal disseminations. Palliative proximal gastrectomy was performed. Pathological examination revealed transmural involvement of DSRCT, and t(11;22)(p12;q12) was demonstrated on fluorescence in situ hybridization test. The chemotherapeutic regimen was changed and the patient underwent 8 additional cycles of post-operative chemotherapy. The patient is now alive and the residual tumor shows no significant changes after chemotherapy.