Intra-abdominal desmoplastic small round cell tumour

Intra-abdominal desmoplastic small blue round cell tumor: A case report

“…Due to its aggressive nature, more than 40% of patients have distant metastases at the time of diagnosis. These are usually located in the liver, lungs, and lymph nodes with a high frequency of paraneoplastic syndrome affecting the patients [7] . The most common symptoms displayed are the presence of abdominal pain, distension, and a palpable abdominal mass.…”

“…The tumor may show a pattern of small, round, oval and/or spindled cells, arranged in nests, cords, lines, sheets and trabeculae with hyperchromatic nuclei, a low volume cytoplasm, an increased mitotic rate, and well-defined solid areas, embedded in a desmoplastic stroma. There are less specific morphological patterns, such as papillary, glandular and cribriform patterns with clear, fusiform, pleomorphic, rhabdoid, and basaloid cells forming rosettes or pseudorosettes [7] .…”

A Classic Presentation of Desmoplastic Small Round Cell Tumor

“…The classical location of DSRCT is intra-abdominal, and while its histogenesis is unknown, a possible mesothelial origin has been proposed based on the predominant involvement of serosae including parietal and visceral peritoneum, pleura, and tunica vaginalis in paratesticular lesions (continuity of peritoneum) [ 4 , 9 ]. Nonetheless, a few reports have described primary lesions in other locations such as the intracranial, sinonasal cavity, salivary glands, as well as colonic, renal, and soft tissue [ 6 ].…”

“…Intracytoplasmic inclusions and even rhabdoid features can be observed in up to 50% of tumor cells [ 13 ]. These cells are predominantly arranged in well-defined nests of varying size, as well as in cords, sheets, trabeculae, solid areas, rosettes, and/or pseudo-rosettes [ 9 ], with extensive necrosis, frequent mitotic figures, cystic degeneration and/or calcification, surrounded by a prominent desmoplastic stroma composed of fibroblasts or myofibroblasts, with loose extracellular material and abundant collagen [ 5 , 12 ]. These morphologic features suggest as differential diagnoses EWS, WT, rhabdomyosarcoma, non-Hodgkin’s lymphoma, synovial sarcoma, leiomyosarcoma, and peritoneal carcinomatosis [ 5 , 13 ].…”

“…Total abdominopelvic radiotherapy (WAP-RT) is a complementary treatment option that has been used with variable results in patients with DSRCT; although new radiotherapy techniques such as intensity-modulated radiation therapy (IMRT) have not achieved a statistically significant improvement in patient survival, they have shown a significant decrease in toxicity, improving tolerance in patients when used as a therapeutic modality after chemotherapy and CCS [ 5 , 9 ]. Despite all the efforts to improve its treatment, DSRCTs still have a poor prognosis, with three- and five-year survival rates below 50% and 20%, respectively [ 5 , 7 , 17 ].…”