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.

Ewing sarcoma: A rare report from the Indian pediatric population

Ewing抯 sarcomas are rare, aggressive tumors with a tendency toward recurrence following resection, and early metastasis. Although patients of younger or older age account for almost 30% of instances, peak incidences occur between the ages of 10 and 20. We, hereby, report the case of a 10-year-old girl who presented with a 3-month history of pain in her right hip that was unable to be relieved by non-steroidal anti-inflammatory medicine and physical therapy. On examination, bone mar

Pituitary blastoma, expanding the spectrum of newly recognized entities. A case report

Tumors of the pituitary gland and sellar region represent about 15% of all brain tumors, with pituitary adenoma being the commonest and pituitary carcinoma being very rare. Pituitary tumors in children are even rarer. Pituitary blastoma, a pediatric adenohypophysial tumor, is a new entity described in the 2017 WHO classification of pituitary tumors. This is a very rare tumor with only 21 cases reported so far. Hence, we are reporting this unusual case seen in a 7-month-old infant who presented with a large sellar/suprasellar mass with pressure symptoms of short duration. Typically, they present between 7–24 months of age. On histopathology, a cellular tumor was seen with primitive-looking round cells with scanty cytoplasm with few well-defined gland or rosette-like structures. The immunohistochemical stains showed diffuse strong staining for synaptophysin with a very high MIB-1 index. Other markers for common round cell tumors in this age group and hormonal markers of pituitary tumors were negative with INI-1 being intact. The initial cases described by Scheithauer presented with Cushing's disease and at least focally expressed adrenocorticotrophic hormone on immunohistochemistry. However, nonfunctioning tumors are also seen, albeit rarely. These are known to be associated with DICER 1 mutations and have a poor prognosis. Hence, morphologic recognition in the right clinical context and excluding other differential diagnoses in infants help make the correct diagnosis.

Neuroblastoma: Application of International Neuroblastoma Pathology Classification on fine needle aspiration cytology smears

Background: Neuroblastoma (NB) is the fourth most common tumor of childhood. There is a paucity of literature on its subtyping of cytology and prognostic utility. Aims: We aimed to study the cytopathological features of NB on the aspirated material, subtype it, and assess the role of International Neuroblastoma Pathology Classification (INPC) classification on cytology smears in the preoperative prognosis of NB. Materials and Methods: Fifteen cases of NB reported on fine-needle aspiration cytology (FNAC) in the past 3 years were included. Detailed clinical, radiological, and cytological features were noted. Smears were assessed for characteristics such as cellularity, neuroblasts (cytoplasmic, nuclear details), rosettes, neuropil, Schwann cells, fibroblasts, calcification, and necrosis. Afterward, cases were categorized as undifferentiated (UD), poorly differentiated (PD), and differentiating (D) subtypes. Mitotic-karyorrhectic index (MKI) was calculated and correlated with histopathology. Follow-up was done to date. Results: The age ranged from 19 days to 10 years with an M: F ratio of 3:1. Twelve cases were retroperitoneal, two cervical, and one mediastinal. Metastatic disease was seen in six cases, one to the cervical, four to the bone marrow, and two to the scalp. The International Neuroblastoma Risk Group (INRG) staging system was available in all cases, out of which three were in stage L1, six in stage L2, four in stage M, and two in stage Ms. On cytology, four cases were differentiating NB, five PD NB, and six UD NB. The MKI was high (>4%) in 80% of UD, intermediate (2–4%) in 100% of PD, and low (<2%) in 75% of D cases. MKI corroborated in both histology and cytology, except in one case. Conclusion: NB can be subtyped on cytology on the basis of characteristics of neuroblasts, presence of neutrophils, rosettes, and necrosis. UD NB has a high MKI and is associated with a poor prognosis. A preoperative comprehensive reporting of NB on cytology can be very useful in guiding appropriate chemotherapy with some increment in survival. However, larger studies are needed to validate the calculation of MKI on FNA smears.

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.

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.

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.

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

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.

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.

Primary primitive neuroectodermal tumor of the kidney

Renal primitive neuroectodermal tumor (PNET) is a rare entity. It should be differentiated from other primary renal malignancies. The differentiation of the malignant small round cell tumors may be challenging; however, it is essential for better management of the patient. We report a 45-year-old male having complaints of pain in the abdomen, hematuria, and mass in the abdomen for 6 months. Abdominal and pelvic sonography finding showed cystic-solid, right renal mass suggestive of malignancy. On histopathology diagnosed as malignant small round blue cell tumor suggestive of primitive neuroectodermal tumor. On immunohistochemistry, CD99 positivity confirmed the diagnosis of primary PNET of the kidney. We are presenting this case for its rarity, clinical presentation, and pathological findings.

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.

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.

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.

"Double Trouble": A case of precocious puberty with concomitant malignant gluteal mass

@#Precocious puberty is the appearance of physical and hormonal signs of pubertal development at an earlier age than is considered normal. The causes may range from a variant of normal development to pathologic conditions such as glands that function abnormally early in life. This is a case of a seven year old female with clitoromegaly, early menarche and a gluteal mass accompanied by elevated 17-hydoxyprogesterone and decreased level of cortisol which supports the diagnosis of congenital adrenal hyperplasia. Gluteal mass cytological features are consistent with a malignant round cell tumor.

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.