Small Round Cell Tumors of the Abdomen and Thorax

Das, Dilip K.; Bhambhani, Suresh; Chachra, Krishan Lal; Murthy, N S; Tripathi, Renu

doi:10.1159/000332785

Cited by 26 publications

(6 citation statements)

References 19 publications

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“…This poses a considerable challenge to the diagnostic skill of the cytopathologist. [37] In fact, a definite diagnosis based on clinical and cytomorphological criteria was possible in 57% of the cases in one study,[9] and in 68% of the cases in another study. [8]…”

Section: Cytomorphological Features Of Msrctmentioning

confidence: 99%

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Malignant small round cell tumors

Rajwanshi

Srinivas

Gautam

2009

J Cytol

131

105

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Malignant small round cell tumors are characterised by small, round, relatively undifferentiated cells. They generally include Ewing's sarcoma, peripheral neuroectodermal tumor, rhabdomyosarcoma, synovial sarcoma, non-Hodgkin's lymphoma, retinoblastoma, neuroblastoma, hepatoblastoma, and nephroblastoma or Wilms’ tumor. Other differential diagnoses of small round cell tumors include small cell osteogenic sarcoma, undifferentiated hepatoblastoma, granulocytic sarcoma, and intraabdominal desmoplastic small round cell tumor. Differential diagnosis of small round cell tumors is particularly difficult due to their undifferentiated or primitive character. Tumors that show good differentiation are generally easy to diagnose, but when a tumor is poorly differentiated, identification of the diagnostic, morphological features is difficult and therefore, no definitive diagnosis may be possible. As seen in several study reports, fine needle aspiration cytology (FNAC) has become an important modality of diagnosis for these tumors. The technique yields adequate numbers of dissociated, viable cells, making it ideally suitable for ancillary techniques. Typically, a multimodal approach is employed and the principal ancillary techniques that have been found to be useful in classification are immunohistochemistry and immunophenotyping by flow cytometry, reverse transcriptase polymerase chain reaction (RT-PCR), fluorescence in situ hybridization (FISH), and electron microscopy. However, the recent characterization of chromosomal breakpoints and the corresponding genes involved in malignant small round cell tumors means that it is possible to use molecular genetic approaches for detection.

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Section: Cytomorphological Features Of Msrctmentioning

confidence: 99%

“…[33738] The presence of strap or tadpole cells is deemed to be strongly associated with rhabdomyosarcoma. [57] The finding of cells with more abundant cytoplasm, eccentrically located nuclei, and bi/multinucleated tumor cells in a background of mucosubstance helps to distinguish RMS from other small round cell tumors. [3940]…”

Section: Rhabdomyosarcomamentioning

confidence: 99%

Malignant small round cell tumors

Rajwanshi

Srinivas

Gautam

2009

J Cytol

131

105

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“…Eosinophilic cytoplasmic inclusion bodies can occasionally be identified [3–5, 19]. Cytological analysis is feasible from tissue biopsy and fine needle aspiration (FNA) specimens [20]. Ascitic and pleural fluid specimens are also of benefit especially if tissue biopsy or FNA specimens are difficult to obtain [20].…”

Section: Discussionmentioning

confidence: 99%

Desmoplastic Small Round Cell Tumor of Stomach

Abu‐Zaid

Azzam

Alnajjar

et al. 2013

Case Reports in Gastrointestinal Medicine

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Desmoplastic small round cell tumor (DSRCT) is an extremely uncommon, highly aggressive, and malignant mesenchymal neoplasm of undetermined histogenesis. Less than 200 case reports have been documented in literature so far. Herein, we report a 26-year-old otherwise healthy female patient who presented with a 1-month history of epigastric pain. On physical examination, a palpable, slightly mobile, and tender epigastric mass was detected. All laboratory tests were normal. A chest, abdominal, and pelvic contrast-enhanced computed tomography (CT) scans showed a 3.8 × 7.2 × 8.7 cm ill-defined mass, involving gastric fundus and extending into gastric cardia and lower gastroesophageal junction. It was associated with multiple enlarged gastrohepatic lymph nodes; the largest measured 1.2 cm. There was no evidence of ascites or retroperitoneal or mesenteric lymphatic metastases. Patient underwent total gastrectomy with D2 lymphadenectomy, splenectomy, and antecolic Roux-en-Y esophagojejunal anastomosis. Histopathological examination revealed coexpression of mesenchymal, epithelial, and neural markers. The characteristic chromosomal translocation (t(11; 22)(p13; q12)) was demonstrated on fluorescence in situ hybridization (FISH) technique. Diagnosis of DSRCT of stomach was confirmed. Patient received no postoperative radiotherapy or chemotherapy. A postoperative 3-month followup failed to show any recurrence. In addition, a literature review on DSRCT is included.

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“…Despite the fact that DSRCT is now a well-documented entity with several cytological reports in the literature, generally comprising case reports that include findings in both FNA material and ascitic/pleural effusions [6,7,8,9,17,18,19,20,21,22], its diagnosis is still usually established by histopathological examination.…”

Section: Discussionmentioning

confidence: 99%

Desmoplastic Small Round Cell Tumor: Diagnosis by Fine-Needle Aspiration Cytology

Leça

Vieira²,

Teixeira³

et al. 2012

Acta Cytologica

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Background: Desmoplastic small round cell tumor (DSRCT) is a distinctive clinicopathologic entity with an aggressive clinical course that typically involves the abdominal and/or pelvic peritoneum of young males. A population of small round blue cells and a fibroesclerotic stroma are the usual morphologic features. This tumor is characterized by a typical polyphenotypic profile with expression of epithelial, mesenchymal and neural markers. Cytogenetically, this tumor presents a unique abnormality – t(11;22)(p13;q12). Case: A 29-year-old male without significant medical history was admitted to our institution with gastrointestinal symptomatology. The physical examination and medical imaging studies revealed an extensive soft tissue mass filling the entire peritoneal cavity/pelvis. A fine-needle aspiration (FNA) of the abdominal mass was performed. The FNA smears revealed fragments of collagenous desmoplastic stroma and clusters of loosely cohesive small round cells that showed positivity for epithelial and myogenic markers. Cytogenetic analysis demonstrated rearrangement of the genes EWSR1 and WT1, resulting from the t(11;22)(p13;q12). Conclusion: DSRCT is an uncommon neoplasm that shares clinical and cytomorphologic features with other small round cell tumors. Therefore, a primary definitive diagnosis based on cytology specimens may be difficult but plausible and can be aided by a typical clinical presentation and ancillary immunocytochemical/cytogenetic studies.

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Small Round Cell Tumors of the Abdomen and Thorax

Cited by 26 publications

References 19 publications

Malignant small round cell tumors

Malignant small round cell tumors

Desmoplastic Small Round Cell Tumor of Stomach

Desmoplastic Small Round Cell Tumor: Diagnosis by Fine-Needle Aspiration Cytology

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