Tumor desmoplásico de células redondas / Desmoplastic round cell tumor

Resumen El tumor desmoplásico de células pequeñas y redon das es una neoplasia poco frecuente y muy agresiva que forma parte de la familia de los "tumores de célu las pequeñas, redondas y azules". Presenta una mayor incidencia en el sexo masculino en la segunda década de la vida. Se debe a la translocación t(11;22) (p13;q12). Se puede localizar tanto en el abdomen como en el re troperitoneo caracterizándose por presentar síntomas inespecíficos. El tratamiento es muy variado y no se ha detectado todavía aquel que garantice la cura total del paciente. El objetivo del presente estudio es exponer un caso clínico de tumor desmoplásico como enfermedad abdo minal infrecuente y su expresión imagenológica.

Abstract Desmoplastic small round cell tumor is a rare and very aggressive neoplasm that belongs to the family of "small round blue cell tumors". It has a higher incidence in males in the second decade of life. It is due to trans location t(11;22) (p13;q12). It can be located both in the abdomen and in the retroperitoneum and is character ized by nonspecific symptoms. The treatment is very varied and the one that guarantees the total cure of the patient has not yet been detected. The objective of this study is to expose a clinical case of desmoplastic tumor as an rare abdominal disease and its imaging expression.

MRI features of desmoplastic small round cell tumor of the abdomen and pelvis / 中华消化外科杂志

Objective:To investigate the magnetic resonance imaging (MRI) features of desmoplastic small round cell tumor (DSRCT) of the abdomen and pelvis.Method:The retrospec-tive and descriptive study was conducted. The clinicopathological data of 8 patients with DSRCT of the abdomen and pelvis, including 3 cases admitted in Yueqing People's Hospital and 5 cases admitted in Wenzhou People's Hospital, from January 2008 to June 2022 were collected. There were 5 males and 3 females, aged (43±5)years. All patients underwent MRI plain and enhanced scanning. Observa-tion indicators: (1) imaging features of DSRCT of the abdomen and pelvis; (2) treatment and pathological examination characteristics of DSRCT of the abdomen and pelvis; (3) follow-up. Measurement data with normal distribution were represented as Mean± SD, and measurement data with skewed distri-bution were represented as M(range). Count data were described as absolute numbers. Results:(1) Imaging features of DSRCT of the abdomen and pelvis. ① Tumor location. Of the 8 patients, there were 6 cases with tumors located respectively at the lower edge of the liver in the right quarter costal region, the medial side of the ileocecal region in the right iliac region, the medial side of the caecum in the right iliac region, the gastro-pancreatic space in the left quarter costal region, the mesenteric space in the left iliac region and the right side of pelvic bladder, and 2 cases with tumors located at retroperitoneal space of left quarter rib region. ② Tumor size. There were 13 lesions in the 8 patients, and the maximum diameter of tumor was 9.1 (range, 3.5?20.0)cm. Of the 8 patients, there were 5 cases with single tumor and 3 cases with multiple tumors. ③ Tumor shape and boundary. Of the 8 patients, there were 4 cases with tumor in expansive growth and 4 cases with tumor in invasive growth. There were 5 cases with tumor of intratumoral necrosis and cystic degene-ration, 4 cases with tumor of intratumoral hemorrhage, 4 cases with tumor of intratumoral spot calcification, 3 cases with tumor of peritumoral tissue exudation. One patient may combined with multiple imaging manifestations. ④ Imaging characteristics of MRI plain scanning. Of the 8 patients, there were 4 cases with tumor of homogeneous hypointensity signal and 4 cases with tumor of hypointensity mixed with speckled hyperintensity (with hemorrhage) in T1 weighted imaging of MRI plain scanning. There were 3 cases with tumor of homogeneous hyperintensity and 5 cases with tumor of high signal at the edge, patchy and spot-shaped in the center in T2 weighted imaging of MRI plain scanning. There were 5 cases with tumor of high, equal and low confounding signals and 3 cases with tumor of high and low signals in T2 weighted imaging fat suppression sequence of MRI plain scanning. There were 3 cases with tumor of uniform high signals and 5 cases with tumor of high, equal and low mixed signals in diffusion weighted imaging of MRI plain scanning. ⑤ Imaging characteristics of MRI enhanced scanning. All 8 patients had tumor of heterogeneous enhancement in MRI enhanced scanning, including 2 cases with significant enhancement in arterial phase, continuous enhancement in portal phase, slightly reduced enhancement in delayed phase, 4 cases with moderate enhancement in arterial phase, continuous enhancement in portal phase, slowly exited enhancement in delayed phase, 2 cases with mild enhancement in arterial phase, continuous enhancement in portal phase, slowly exited enhancement in delayed phase. Of the 8 patients, there were 3 cases with tumor of annular enhancement with intratumoral strip or grid signals and 3 cases with tumor of peritumoral blood vessels increased and thickened signals. ⑥ Tumor invasion and metastasis. Of the 8 patients, there were 4 cases with tumor invaded bowel, 2 cases with tumor invaded surrounding tissues, 1 case with tumor invaded left kidney, spleen and pancreatic tail, 1 case with tumor invaded distal of left ureter. There were 5 cases with abdominal, retroperitoneal and inguinal lymph nodes enlargement, 4 cases with multiple nodular thickening of peritoneum and ascites, 2 cases with tumor liver and lung metastasis and 1 case with tumor rib, femur and sacrum metastasis. One patient may combined with multiple tumor metastasis. (2) Treatment and patholo-gical examination characteristics of DSRCT of the abdomen and pelvis. Of the 8 patients, 3 patients underwent complete resection as clear tumor boundary, 3 patients underwent tumor partial resection as tight adhesion between tumor and surrounding blood vessels, 2 cases underwent tumor tissue pathological examination as extensive metastasis of peritoneum, omentum, mesentery and surrounding intestine. All 8 patients were diagnosed as DSRCT by microscopic examination, electron microscopic examination, immunohistochemical staining and cytogenetic examination. (3) Follow-up. All 8 patients underwent postoperative follow-up and died during the follow-up.Conclusion:MRI features of abdominal and pelvic DSRCT include single or multiple lobulated masses with unclear boundaries, invading the omentum, mesentery, peritoneum and adjacent tissues in most cases, mixed signals and heterogeneous mild to moderate enhancement in enhanced scanning.

Omental desmoplastic small round cell tumor with metastasis

Desmoplastic small round cell tumor (DSRCT) is a very rare diagnosis with about 200 cases reported in literature. DSRCT is a recently described histopathological entity by Gerald and Rosai in 1989. Abdominopelvic cavity especially peritoneum is the most common site. We report a case of a huge omental DSRCT with lymph node metastasis which was initially misdiagnosed as gastrointestinal stromal tumor on radiology. A 26-year-old male presented with complaints of upper abdominal swelling associated with constant dull pain. On examination there was a large 15 × 12 cm intraabdominal mass in the epigastric and umbilical region. Imaging studies were suggestive of neoplastic mesenchymal etiology. Image-guided fine-needle aspiration cytology (FNAC) was suggestive of mesenchymal neoplastic etiology. On laparotomy, there was a huge 20 × 15 cm mass arising from omentum with multiple omental and mesenteric seedlings and mesenteric, peripancreatic and perigastric lymphadenopathy. The patient underwent debulking surgery with uneventful post-operative recovery. Histopathological examination with immunohistochemistry revealed a diagnosis of DSRCT of omentum and small bowel mesentery with lymph node metastasis. Patient then received adjuvant chemotherapy with multiple chemotherapeutic drugs as per P6 protocol and has stable disease at 1 year follow up.

Therapeutic targets of desmoplastic small round cell tumor and future direction / 中国肿瘤临床

Desmoplastic small round cell tumor (DSRCT) is a rare and highly malignant soft tissue sarcoma. Most patients are diagnosed at advanced stage unless DSRCT happens to be discovered accidentally. DSRCT mainly occurs in the abdomen and pelvis, spreading along the peritoneal surface. At the time of diagnosis, most patients have passed the operable stage. The diagnosis of DSRCT is based on the histological analysis of a biopsy. It typically manifests as small round blue cells in the nest, separated by a large number of fibroproliferative stroma. A stable cytogenetic feature of DSRCT is that the characteristic t(11; 22) (P13; Q12) chromosome produces the EWSWT1 fusion gene. The prognosis of patients with DSRCT is very poor, and the 5-year survival rate is about 15%. Despite the use of active treatment methods, such as chemotherapy, surgery, and total abdominal radiotherapy, about 60% 70% of patients with DSRCT die within 2- 3 years. Targeted therapy, immunotherapy, and other methods have been trialed recently in the treatment of DSRCT due to the development of DSRCT molecular genetics.

Eight children with desmoplastic small round cell tumor and literature review / 中华实用儿科临床杂志

Objective:To analyze the clinical characteristics, therapeutic modalities and prognosis of desmoplastic small round cell tumor (DSRCT) in children, and to summarize the international research progress.Methods:A total of 8 children with DSRCT admitted to Shanghai Children′s Medical Center, Shanghai Jiaotong University, School of Medicine, from January 1999 to August 2019 were retrospectively studied.The clinical characteristics, consultation process and follow-up results were summarized, and the Kaplan-Meier survival analysis method was used to calculate the survival rate.Results:Among these 8 cases, there were 6 male children and 2 female children.Seven cases originated in the abdomen and pelvis, and 1 case originated in the sacral region.All cases had infiltrate surrounding tissues or viscera, and 4 cases(50%) had extra-peritoneal metastasis, including distant lymph node metastasis, liver, lung and bone metastasis.All patients received chemotherapy, among which 3 patients received radiotherapy, and 2 patients received autologous hematopoietic stem cell transplantation.The medical follow-up was continued to February 15, 2020, with the median follow-up period being 59 months.Three cases died and 5 cases survived (2 cases in complete remission, 1 case in recurrent relapse, 2 cases in partial remission still under treatment). The median relapse time was 14.5 months, the 3-year relapse-free survival rate was (30.0±17.5)%, and 3-year overall survival was (51.4±20.4)%.Conclusions:Half of DSRCT had distant metastasis; the prognosis was poor despite the aggressive multimodality therapeutic approaches, such as chemotherapy, cytoreductive surgery, and whole abdominopelvic radiotherapy and stem cell transplantation.

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.

Chemotherapy combined with apatinib for the treatment of desmoplastic small round cell tumors: A case report

Desmoplastic small round cell tumor (DSRCT) is a type of soft-tissue sarcoma with poor prognosis. Current treatments include multidisciplinary treatment options such as surgery, chemotherapy, and radiotherapy. Apatinib is an oral, small-molecule, anti-tumor, angiogenesis-targeted drug, which acts mainly on the intracellular binding site of vascular endothelial growth factor receptor-2. In this study, we administered apatinib in combination with chemotherapy to achieve good disease control. This is a 31-year-old male who presented with upper abdominal pain, nausea, and anorexia for over a month. Imaging revealed multiple solid masses and ascites in the liver and abdominal cavity. He was diagnosed as having cholangiocarcinoma with metastasis to the liver, both lungs, bone, and multiple lymph nodes in the neck, abdominal and pelvic cavity, retroperitoneum, and palpitate angle, based on a percutaneous biopsy of the liver and an abdominal mass, and other examinations. Computed tomography revealed disease progression after two cycles of gemcitabine combined with nedaplatin chemotherapy. Next-generation sequencing detection based on the Illumina high-throughput sequencing platform suggested EWSR1 exon7- Wilms tumor 1 exon8 fusion. The pathology was verified and diagnosed as DSRCT. The chemotherapy regimen was changed to cyclophosphamide, epirubicin, vincristine, and oral apatinib for two cycles. The lesions were mostly reduced, and partial response was evaluated. This case is the first report of the efficacy of apatinib combined with systemic chemotherapy in the treatment of DSRCT, which can become an alternative treatment for this disease

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.

FLI1 and MIC2 expression in precursor B-lymphoblastic leukemia with Burkitt-like morphology and extensive extramedullary involvement: A diagnostic challenge in pediatric small round cell tumor

Pediatric small round cell tumors (PSRCTs) constitute a large proportion of childhood malignancies with overlapping diagnostic and clinical features but radically different therapies. Here, we report a case of 16-year-old male child presenting with diffuse abdominal and mediastinal mass, axillary lymphadenopathy, and pleural effusion. Bone marrow aspirate showed near total replacement by small round malignant cells. The bone marrow biopsy showed interstitial infiltration by malignant cells, which were CD45? CD3? CD20? MIC2+ FLI1+ and diagnosis of Ewing's sarcoma was established. In contrast, flowcytometric immunophenotyping of the bone marrow aspirate showed CD45? cells, which were CD19+ cytCD79a+ CD10+ CD81+ CD38+ HLA-DR+ CD22+ CD20? consistent with B-cell acute lymphoblastic leukemia (B-ALL). The extended immunostaining panel on bone marrow biopsy also showed positivity for cytCD79a, CD10, CD19, and BCL-2, whereas fluorescent in-situ hybridization for EWSR1 gene rearrangement was negative. Thus, a final diagnosis of CD45? FLI1+ MIC2+ B-ALL was established. Rare cases of CD45? B-ALL with immunoreactivity for MIC2 and Friend leukemia virus integration 1 (FLI1) have posed a diagnostic challenge for PSRCTs in the recent past. This case report highlights the role of multimodality approach in establishing a correct diagnosis in CD45? PSRCTs to ensure definitive therapy and better clinical outcome.

Small round cell lesions of the bone: Diagnostic approach, differential diagnoses and impact on treatment

Small round cell lesions of the bone encompass a heterogeneous group of tumors and tumor-like lesions, including Ewing sarcoma, small cell osteosarcoma, mesenchymal chondrosarcoma, neuroblastoma, non-Hodgkin's lymphoma (NHL), “Ewing-like” undifferentiated round cell sarcomas, metastasizing small cell carcinoma, along with plasma cell dyscrasia and Langerhan's cell histiocytosis. At the same time, there are tumor mimics, for example, chronic osteomyelitis, which has overlapping radiologic features with Ewing sarcoma and a primary intraosseous NHL. An exact diagnosis necessitates integration of clinical, radiologic, pathologic, and ancillary test results, including immunohistochemical and molecular results. Currently, there are several immunohistochemical markers and specific molecular signatures, driving most of these tumors, available, for an exact diagnosis. This review focuses on a pragmatic approach towards uncovering specific small round cell lesions of the bone, emphasizing upon integration of traditional morphology with ancillary techniques, including immunohistochemical markers and molecular techniques, the latter, especially in cases of Ewing sarcoma, Ewing-like undifferentiated round cell sarcoma, mesenchymal chondrosarcoma, and neuroblastoma. Subsequent to the diagnostic approach, including an impact on treatment, individual intraosseous round cell lesions have been described in detail. The references include updated articles from PUBMED.

A case report and literature review of Abdominal Desmoplastic Small Round Cell Tumor / 中国综合临床

Objective To investigate the pathological characteristics,diagnosis,treatment decision and short?term curative effect of abdominal desmoplastic small round cell tumor??Methods The clinical data of a case of desmoplastic small round cell tumor admitted to Gansu provincial people′s Hospital in April 2018 were analyzed retrospectively??The clinical manifestations, pathological features, diagnosis and differential diagnosis, treatment and prognosis of desmoplastic small round cell tumor were summarized and analyzed??Results The patient was successfully treated with maximum tumor reduction??The operation time was 360 minutes??The estimated blood loss during operation was 200 ml,and no blood was transfused during operation??The abdominal drainage tube was removed on the 8th day after operation and the liver function recovered well??Postoperative pathology: ( retroperitoneal) small round cell malignant tumor??Combined with clinical and immunohistochemical staining results: highly considered: desmoplastic small round cell tumor??The patient was discharged on the 16th day after operation??The patient was followed up for 4 months and the tumor recurred and liver metastasis??The follow?up period is now up to October 2018??Conclusion Desmoplastic small round cell tumor is a rare and highly malignant soft tissue small cell tumor with poor prognosis??Imaging examination and detection of tumor markers have no specificity and diagnose of it is difficult??Complete resection of the tumor and combined chemotherapy can improve the prognosis of the patients,but the prognosis is still not satisfactory, and more effective treatment decisions still need to be explored??

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.

Desmoplatic Small Round Cell Tumor Of Paratesticular Origin

Desmoplastic Small Round Cell Tumor (DSRCT) of the paratesticular region is an extremely rare mesenchymal tumor occurring in adolescence with a tendency for extensive metastases. We report a rare case of DSRCT of paratesticular origin in a 24 year old male who presented with painless right testicular mass and disseminated abdominal disease. The patient underwent right inguinal orchidectomy and on Histopathology and Immunohistochemistry the diagnosis was confirmed. The patient was treated with multi agent chemotherapy with partial remission. Prognosis of such patients is generally dismal despite multimodality treatment.

Advances of diagnosis and therapy for desmoplastic small round cell tumor / 国际肿瘤学杂志

Desmoplastic small round cell tumor (DSRCT) is a rare and high malignant soft tissue tumor with very poor prognosis.It usually occurs in the abdominopelvic cavity of adolescents and young males.DSRCT is prone to occur distant metastasis,mainly in the liver and lung.The histopathological manifestation is featured with nests of small round blue cells separated by desmoplastic stroma.DSRCT can co-express epithelial,neural and mesenchymal markers.The molecular characteristic of DSRCT is the production of EWSWT1 fusion protein via the translocation of chromosome t (11;22) (p13;q12).Treatments of DSRCT include radical resection or cytoreductive surgery,high intensity systemic chemotherapy,local radiotherapy and hyperthermic intraperitoneal chemotherapy.

Analysis of clinical features and prognostic factors of desmoplastic small round cell tumor / 上海交通大学学报（医学版）

Objective: To analyze the clinical features and prognostic factors of desmoplastic small round cell tumor (DSRCT). Methods: Clinical datum of 140 patients with DSRCT published from Nov. 2003 to Jul. 2012 were collected and studied retrospectively by searching Medline and Embase databases. The observation indicators were progression-free survival (PFS) or overall survival (OS). Survival rates were calculated using the Kaplan-Meier method and compared between groups using a log-rank test. Multivariate analysis was performed using the Cox model to determine the prognostic factors. Results: Patient median age was (23.2±12.7) years (range 4-74 years, the ratio of male and female was 3.12: 1). Frequent symptoms were abdominal pain (35.7%) and evidence of a palpable mass (20.0%). 106 cases tumors were in the abdominal or pelvic cavity, the remaining were extra-abdominal tumors. The frequency of patients receiving conventional chemotherapy, cytoreductive surgery, neoadjuvant chemotherapy, adjuvant chemotherapy or first-line chemotherapy was 76.4%, 52.1%, 17.1%, 47.9% and 38.6%, respectively. Some patients received adjuvant radiotherapy (17.1%), hyperthermic intraperitoneal chemotherapy (4.1%) and bone marrow transplantation (7.3%). By univariate analysis, male gender, absence of metastasis, effective cytoreductive surgery, chemotherapy and multimodal therapy were significant prognostic factors for prolonged OS (all P<0.05). Primary tumor site, extraabdominal tumors, absence of metastasis and effective cytoreductive surgery were associated with improved PFS (all P<0.05). Cox regression analysis showed effective cytoreductive surgery and chemotherapy were independent prognostic factors. Conclusion: Multimodal therapeutics that clear tumors by surgery, adjuvant therapy are favorable prognostic factors for improved survival level in DSRCT patients.

Analysis of clinical features and prognostic factors of desmoplastic small round cell tumor / 上海交通大学学报（医学版）

Objective·To analyze the clinical features and prognostic factors of desmoplastic small round cell tumor (DSRCT). Methods·Clinical datum of 140 patients with DSRCT published from Nov. 2003 to Jul. 2012 were collected and studied retrospectively by searching Medline and Embase databases. The observation indicators were progression-free survival (PFS) or overall survival (OS). Survival rates were calculated using the Kaplan-Meier method and compared between groups using a log-rank test. Multivariate analysis was performed using the Cox model to determine the prognostic factors. Results·Patient median age was (23.2±12.7) years (range 4-74 years, the ratio of male and female was 3.12:1). Frequent symptoms were abdominal pain (35.7%) and evidence of a palpable mass (20.0%). 106 cases tumors were in the abdominal or pelvic cavity, the remaining were extra-abdominal tumors. The frequency of patients receiving conventional chemotherapy, cytoreductive surgery, neoadjuvant chemotherapy, adjuvant chemotherapy or first-line chemotherapy was 76.4%, 52.1%, 17.1%, 47.9% and 38.6%, respectively. Some patients received adjuvant radiotherapy (17.1%), hyperthermic intraperitoneal chemotherapy (4.1%) and bone marrow transplantation (7.3%). By univariate analysis, male gender, absence of metastasis, effective cytoreductive surgery, chemotherapy and multimodal therapy were significant prognostic factors for prolonged OS (all P<0.05). Primary tumor site, extra-abdominal tumors, absence of metastasis and effective cytoreductive surgery were associated with improved PFS (all P<0.05). Cox regression analysis showed effective cytoreductive surgery and chemotherapy were independent prognostic factors. Conclusion·Multimodal therapeutics that clear tumors by surgery, adjuvant therapy are favorable prognostic factors for improved survival level in DSRCT patients.

Adult Onset Sinonasal Rhabdomyosarcoma - A Rare Case Report With Cytohistological Features

Rhabdomyosarcoma (RMS) is a fast growing, malignant tumour arising from immature mesenchymal cells, committed to skeletal muscle differentiation. It is more often seen in the paediatric population and constitutes less than 1% of all malignancies and less than 3% of all soft tissue tumours. RMS of the paranasal sinuses constitutes 10-15 % of adult head and neck RMS, ethmoidal and maxillary sinuses being the most common. We report a 56-year-oldman presenting with left nasal obstruction, epistaxis on and off and left cheek swelling. Nasal endoscopy revealed a reddish friable mass, bleeding on touch, in the left nasal cavity. CECT scan showed a heterogeneous growth in the left maxillary sinus eroding the medial orbital wall and lateral nasal wall. FNAC of the left cheek swelling yielded highly cellular smears showing predominantly singly scattered round to ovoid neoplastic cells with scanty cytoplasm and indistinct nucleoli. Few of the cells had eccentric nuclei with moderate amount of eosinophilic cytoplasm. Attempted pseudorossette formation was seen. An impression of round cell tumour was given. A diagnosis of an adult onset sinonasal rhabdomyosarcoma was made on histopathological examination of the nasal biopsy, supported by immunohistochemistry (IHC) showing strong myogenin positivity, focal positivity for PAX8 and negativity for CK, LCA, S-100 and CD99. Parameningeal RMS is rare in adults especially the elderly. However, it needs to be considered whenever a poorly-differentiated neoplasm is seen in this age and IHC is a useful aid.

Diagnosing A Rare Case Of Desmoplastic Small Round Cell Tumour On Liver Biopsy

A 50-year-old male of Indian descent presented with jaundice and right hypochondrium pain. Following a computed tomography (CT) scan of the abdomen, a segment 7 liver lesion was visualized, accompanied by extensive peritoneal tumour deposits. An ultrasound guided liver biopsy was performed and histology showed loose nests and sheets of tumour cells with a small blue round cell morphology. The tumour cells showed patchy strong immunopositivity for cytokeratins (AE1/3, CK7, CK19) and synaptophysin, while showing diffuse strong perinuclear positivity for desmin. Interphase fluorescence in-situ hybridization (FISH) study using EWSR1 breakapart probe was positive for EWSR1 gene rearrangement. Desmoplastic small round cell tumour is a rare but aggressive intra-abdominal mesenchymal tumour. While the primary sites of involvement are usually the peritoneum and omentum, visceral involvement can occur. We wish to highlight the importance of considering this entity when evaluating a liver biopsy especially in a less than classical clinical context.

Value of apparent diffusion coefficient in the differential diagnosis of small round cell malignant tumors of nasal and paranasal sinus / 中华放射学杂志

Objective To investigate the diagnostic value of ADC in the evaluation of small round cell malignant tumors(SRCMT) of nasal and paranasal sinus.Methods This study included 143 patients with surgically confirmed SRCMT and Non-SRCMT of nasal and paranasal sinus between 2008 and 2015, all patients underwent diffusion weighted MRI at 3.0 T with a b factor of 0 and 1 000 s/mm2.Quantitative analysis of ADC values was performed.Difference in ADC values between SRCMT and Non-SRCMT was evaluated using the independent samples t test.One-way analysis of variance(ANOVA) test was performed to compare the ADC values of SRCMT.Receiver operating curves (ROC) were developed to determine the cutoff points to differentiate SRCMT from Non-SRCMT.Results There were 98 SRCMT, of which 20 lesions were rhabdomyosarcoma(RMS), 19 lesions were non-Hodgkin's lymphoma(NHL), 4 lesions were malignant melanoma(MM), 14 lesions were neuroendocrine carcinoma(NEC), 12 lesions were Ewing sarcoma or primitive neuroectodermal tumor(EWS or PNET), 11 lesions were extramedullary plasmacytoma(EMP), and 8 lesions were olfactory neuroblastoma(ON).There were 45 Non-SRCMT, of which 28 lesions were squamous cell carcinoma(SCC) and 17 lesions were adenoid cystic carcinoma(ACC).The mean ADC value of SRCMT[(0.66 ± 0.12) × 10-3mm2/s] was significantly different (t=14.97, P＜0.01) from Non-SRCMT [(1.02± 0.16)× 10-3mm2/s].All of 7 kinds of SRCMT were divided into 3 groups according to ADC values: NHL,MM, NEC,EMP;RMS,EWS,PNET;ON.There was statistically significant difference among all 3 groups(F=39.743, P＜0.01), and the differences between any 2 groups were still statistically significant.The area under the ROC of ADC values diagnosing SRCMT was 0.975.Compared with pathological results, an ADC value of 0.82 × 10-3mm2/s was used as the threshold for diagnosing SRCMT with a sensitivity of 97.8％ (44/45),specificity of 89.8％(88/98), and accuracy of 92.3％ (132/143).ADC value had high correlations compared with pathological results (Kappa value was 0.831).Conclusion The ADC value is a non-invasive imaging parameter that can be used to effectively assess SRCMT of nasal and paranasal sinus.

A case report of transverse colon desmoplastic small round cell tumor / 实用肿瘤学杂志

Desmoplastic small round cell tumor( DSRCT) is a kind of highly uncommon malignant tumor which is reported in very recent years.Since it has been firstly reported by Gerald in 1991,DSRCT has only a-round one hundred reported cases globally, and very limited cases domestically.This report illustrates the first case of DSRCT in our hospital so as to share and to discuss with clinicians.