Desmoplastic Small Round Cell Tumors: Cytologic, Histologic, and Immunohistochemical Features

2006 ◽  
Vol 130 (5) ◽  
pp. 728-732 ◽  
Author(s):  
Fuju Chang
Keyword(s):  
Differential Diagnosis ◽  
Abdominal Cavity ◽  
Cell Tumor ◽  
Round Cell ◽  
Cytologic Diagnosis ◽  
Small Round Cell ◽  
Karyotypic Abnormality ◽  
The Family ◽  
Cytogenetic Profile ◽  
Round Cell Tumors

Abstract Desmoplastic small round cell tumor (DSRCT) is a recently recognized clinicopathologic entity that has a predilection for adolescent males and usually affects the abdominal cavity. Due to its uncommon nature, many pathologists lack experience with this tumor. The literature regarding DSRCT is reviewed with special attention to its histologic and cytologic diagnosis. Morphologic features of DSRCT and its immunohistochemical and cytogenetic profile are summarized and differential diagnosis with other small round cell tumors is discussed. As observed by both histologic and cytologic examinations, small round blue cells and fibrosclerotic stroma are the striking morphologic features of DSRCT. The typical immunohistochemical profile is characterized by coexpression of epithelial, mesenchymal, myogenic, and neural markers. Cytogenetically, this tumor harbors a specific karyotypic abnormality, namely t(11;22)(p13;q12). These features distinguish DSRCT from other members of the family of small round cell tumors.

Desmoplastic Small Round Cell Tumors With Atypical Presentations: A Report of 34 Cases

2018 ◽  
Vol 27 (3) ◽  
pp. 236-243 ◽  
Author(s):  
Alyaa Al-Ibraheemi ◽  
Cory Broehm ◽  
Munir R. Tanas ◽  
Andrew E. Horvai ◽  
Brian P. Rubin ◽  
...  
Keyword(s):  
Abdominal Cavity ◽  
Small Cell ◽  
Cell Tumor ◽  
Round Cell ◽  
Cell Sarcoma ◽  
Young Males ◽  
Small Round Cell ◽  
Atypical Presentations ◽  
Round Cell Tumors ◽  
Head Neck

Objectives. Desmoplastic small round cell tumor (DSRCT) is an aggressive round cell sarcoma that arises in the abdominal cavity/pelvis of young males. We sought to expand its clinicopathologic spectrum. Methods. Cases of DSRCT presenting in patients >30 years of age or tumors arising outside of the abdominal cavity/pelvis were retrieved. Results. Thirty-four cases were identified. Sixteen tumors arose at atypical sites (head/neck, intracranial, thigh, axilla/shoulder, inguinal/paratesticular, intraosseous, and uterine corpus). The remaining 18 patients were older than 30 years, and their tumors involved the abdomen or pelvis. The majority of cases showed areas with classic histology, while 6 cases exhibited solid growth and 5 showed macronodular architecture. Cytologic appearance included round cell, rhabdoid, epithelioid, and small cell. Conclusion. DSRCT may arise at nonabdominal locations in both pediatric and adult populations, as well as intra-abdominally in older adults, and these tumors exhibit high rates of metastasis and morbidity.

scholarly journals Desmoplastic small round cell tumor: A case report of a rare differential diagnosis of solid tumors of the pleura

2015 ◽  
Vol 10 (5) ◽  
pp. 2991-2995 ◽  
Author(s):  
YUAN CAO ◽  
YING CHEN ◽  
LI YANG ◽  
ZI-HUA QIAN ◽  
SHU-GAO HAN ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Case Report ◽  
Solid Tumors ◽  
Cell Tumor ◽  
Round Cell ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
Rare Differential Diagnosis

Desmoplastic Small Round Cell Tumor

2019 ◽  
Author(s):  
James A Saltsman III ◽  
Todd E Heaton
Keyword(s):  
Abdominal Cavity ◽  
Local Therapy ◽  
Residual Tumor ◽  
Cell Tumor ◽  
Clinical Approach ◽  
Round Cell ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
Consensus Statements

Desmoplastic small round cell tumor (DSRCT) is a relatively recently identified, rare, aggressive cancer that arises from the peritoneal lining of the abdominal cavity and predominantly affects male adolescents and young adults. DSRCT is included in the Ewing family of tumors and harbors a unique translocation between EWSR and WT1 genes. Patients characteristically present with advanced disease, including widespread involvement of the abdominal cavity and pelvis, with frequent extraperitoneal metastases involving liver, spleen, and thoracic lymph nodes. Five-year overall survival remains approximately 15 to 20% in recent series despite aggressive multimodal therapy. Neoadjuvant chemotherapy, complete resection, defined as less than 1 cm3 of residual tumor, and consolidative whole abdominopelvic radiotherapy (WAP-RT) appear to play important role in curative treatment. The rarity of DSRCT makes large randomized trials difficult and consensus statements about clinical approach impossible. However, this chapter summarizes the best available data on the pathogenesis, diagnosis, and treatment of DSRCT; highlights the major advances made in these areas; and discusses the addition of local therapy to the treatment paradigm. This review contains 3 figures, 1 table, and 51 references.   Key Words: DSRCT, peritoneal malignancy, desmoplastic round cell tumor, surface malignancies of the peritoneum, desmoplastic small round cell tumor of the abdomen, surgical oncology, rare malignancies, surgical therapy of abdominal malignancies

Clinical, pathologic, and molecular spectrum of tumors associated with t(11;22)(p13;q12): desmoplastic small round-cell tumor and its variants.

1998 ◽  
Vol 16 (9) ◽  
pp. 3028-3036 ◽  
Author(s):  
W L Gerald ◽  
M Ladanyi ◽  
E de Alava ◽  
M Cuatrecasas ◽  
B H Kushner ◽  
...  
Keyword(s):  
Tumor Cells ◽  
Abdominal Cavity ◽  
Neuron Specific Enolase ◽  
Chimeric Protein ◽  
Cell Tumor ◽  
Round Cell ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
Translocation Breakpoints

PURPOSE Intense investigation has reshaped concepts about undifferentiated tumors occurring in young people (small round-cell tumors). Tumors associated with t(11;22)(p13;q12) and descriptively designated desmoplastic small round-cell tumor (DSRCT) are a distinctive, rare, poorly understood member of this family. We reviewed 109 cases of DSRCT to further characterize this entity better. METHODS Clinical information and histology were reviewed. Immunohistochemistry and immunoblotting were performed using standard techniques. Chimeric EWS-WT1 RNA and DNA were detected by polymerase chain reaction (PCR) and genomic translocation breakpoints mapped in a subset of cases. RESULTS There were 90 males and 19 females from 6 to 49 years of age (mean, 22 years). A total of 103 had tumor in the abdominal cavity, four in the thoracic region, one in the posterior cranial fossa, and one in the hand. Typical histologic and immunohistochemical features were usually evident in well-sampled tumors, but variations in cellularity, stromal components, cytology, architecture, and immunoreactivity occurred. Tumor cells were usually reactive with antibodies to keratin (67 of 78 cases, 86%), epithelial membrane antigen (50 of 54, 93%), vimentin (64 of 66, 97%), desmin (70 of 78, 90%), neuron-specific enolase (60 of 74, 81%), and the EWS-WT1 chimeric protein (25 of 27, 93%); typically nonreactive for muscle common actin (one of 58, 2%), myogenin (zero of eight, 0%), and chromogranin (one of 46, 2%); and variably reactive for MIC2 (nine of 47, 20%) and p53 (five of 17 with > 20% tumor cells reactive). Functional EWS-WT1 gene fusion was evident in 25 of 26 cases with genomic breakpoints in WT1 intron 7, and EWS introns 7, 8, and 9. Prognosis in general is poor, but tumors are responsive to aggressive therapy. CONCLUSION This large review identifies a greater degree of clinical, pathologic, and molecular variation than originally appreciated for tumors associated with t(11;22)(p13;q12). Translocation and functional fusion of the EWS and WT1 genes appears to be a consistent feature of this unique tumor.

Desmoplastic Small Round Cell Tumor: A Rare but Aggressive Tumor in Young Women

2016 ◽  
Vol 8 (3) ◽  
pp. 239-242
Author(s):  
K Harish ◽  
G Nandini ◽  
K Padma ◽  
ACV Swamy ◽  
Murali Subramanian
Keyword(s):  
Young Women ◽  
Abdominal Cavity ◽  
Malignant Neoplasm ◽  
Cell Tumor ◽  
Round Cell ◽  
Female Ratio ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
Aggressive Tumor

ABSTRACT Desmoplastic small round cell tumor (DSRCT) is a rare malignant neoplasm. It is primarily found in young men, with a reported male to female ratio of 4:1. The tumor typically develops in the abdominal cavity, invading the omentum with multiple peritoneal implants involving the diaphragm, splenic hilum, mesentery of the small and large bowel, and the pelvic peritoneum. Most of them have widespread disease at presentation, with an organ of origin difficult to ascertain. We report a case of desmoplastic round cell tumor in a 17-year-old teenage girl. Immunohistochemistry helped in the diagnosis. She received multimodality treatment. How to cite this article Nandini G, Harish K, Swamy ACV, Subramanian M, Padma K. Desmoplastic Small Round Cell Tumor: A Rare but Aggressive Tumor in Young Women. J South Asian Feder Obst Gynae 2016;8(3):239-242.

scholarly journals Desmoplastic small round cell tumor: review of therapy including surgery followed by continuous hyperthermic peritoneal perfusion of chemotherapy

2011 ◽  
Vol 3 (3) ◽  
pp. 195
Author(s):  
Andrea Hayes-Jordan ◽  
Peter Anderson
Keyword(s):  
Residual Disease ◽  
Fusion Gene ◽  
Abdominal Cavity ◽  
Cell Tumor ◽  
Round Cell ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
Peritoneal Perfusion ◽  
Microscopic Residual Disease

Desmoplastic small round cell tumor (DSRCT) is a very rare disease of children, adolescents, and young adults and involves the abdominal cavity. DSRCT has characteristic fusion gene involving EWS1 and WT1 translocation, t(11;22)(p13;q12). Unlike Ewing’s sarcoma of bone, DSRCT usually presents with diffuse peritoneal implants that are prone to recur. The primary organ of origin of DSRCT is mesenchyme of the peritoneum. This makes it a very unique tumor that is difficult to treat because of the infiltrative and diffuse nature of the peritoneum. The challenge of local control is to remove dozens to hundreds of tumors studding the peritoneal cavity, and then eliminate microscopic disease. We review a sequential multimodality strategy to reduce macroscopic and microscopic disease including neoadjuvant chemotherapy, aggressive surgery including an emerging new therapy to use after surgery to treat microscopic residual disease: continuous hyperthermic peritoneal chemotherapy,

Small Round Cell Tumors

1977 ◽  
Vol 35 ◽  
pp. 528-529
Author(s):  
B. Mackay ◽  
D. Garza
Keyword(s):  
Cell Tumor ◽  
Round Cell ◽  
Cell Type ◽  
Clinical Behavior ◽  
Specific Diagnosis ◽  
Small Round Cell ◽  
Heterogenous Group ◽  
Architectural Organization ◽  
Round Cell Tumors ◽  
Selection Of

The term "small round cell tumor" is used to designate a heterogenous group of neoplasms that have in common the fact that they are composed of small round, polygonal, or occasionally ovoid cells, compactly grouped with little or no evidence of architectural organization. Identification of tumors within this group by light microscopy can be difficult and is often not possible. Selection of a specific diagnosis by the pathologist is frequently influenced by the clinical behavior and location of the tumor, and by the age of the patient. For example, tumors with this morphology occurring in young children are usually assumed to be rhabdomyosarcoma, neuroblastoma, Ewing's sarcoma, or lymphoma.By studying these neoplasms with the electron microscope and correlating the findings with the light microscopic and clinical data, it is possible to reach a specific diagnosis in most cases. Each tumor possesses distinctive ultrastructural features that serve to identify the cell type.

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