A Case Report on Malignant Rhabdoid Round Cell Tumor in Pelvis

The malignant neoplasm is called as malignant rhabdoid tumor or renal tumor. The Malignant tumor had the highest rate of proliferation. Tiny, round, and generally undifferentiated cells make up malignant small round cell tumours. A round cell tumor is a group of malignant tumors composed of relatively small and undifferentiated cells with an increased nuclear - cytoplasmic ratio. Soft tissue malignant tumours of the abdomen and pelvis are a rare but serious kind of cancer. Examples of these tumours include Ewing's sarcoma, peripheral neuroectodermal tumour, rhabdomyosarcoma, synovial sarcoma, non-lymphoma, Hodgkin's retinoblastoma, neuroblastoma, and hepatoblastoma. Mast cell tumour, histiocytoma, lymphoma, plasmacytoma, and transmissible venereal tumours are some of the different types of round cell tumours. Melanomas are the cytologic "great impostor," as they might look on cytology as round cell tumours despite being classed as mesenchymal cancers. Rhabdoid tumours have long been thought to be extremely malignant and have a bad prognosis. Children with this form of tumour have a six to eleven-month median survival duration. They're also less common. In 5% of small round cell tumor patients, can be curable, and it is best achieved by combining systemic chemotherapy with thorough cytoreductive surgery. Here we report a 9-year-old female child who was diagnosed with a malignant rhabdoid round cell tumor in the pelvis. She has undergone excision of pelvic floor tumor andfurther managed with Chemotherapy.

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

Journal of SAFOG with DVD ◽

10.5005/jp-journals-10006-1425 ◽

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.

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DESMOPLASTIC SMALL ROUND CELL TUMOR: A CASE REPORT

10.36106/ijsr/6203210 ◽

2021 ◽

pp. 7-8

Author(s):

Bhuwan Kumar ◽

Rajde Singh ◽

Anurag Mishra

Keyword(s):

Young Adults ◽

Case Report ◽

Poor Prognosis ◽

Malignant Neoplasm ◽

Cell Tumor ◽

Round Cell ◽

Round Cell Tumor ◽

Small Round Cell Tumor ◽

Small Round Cell ◽

Mesenchymal Tissue

Desmoplastic small round cell tumor is a rare, highly malignant neoplasm originating from mesenchymal tissue which was initially described in 1 1991 by Gerald and Rosai. It is composed of small round tumor cells of uncertain histogenesis associated with prominent stromal desmoplasia and 2 polyphenotypic differentiation. It typically occurs in adolescents and young adults. Usually presents with widespread abdominal, serosal, and mesenteric involvement with poor prognosis.

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Multimodal treatment of an extremely rare desmoplastic small round cell tumor primary to the brachial plexus – A case report and review of literature

Surgical Neurology International ◽

10.25259/sni-291-2019 ◽

2019 ◽

Vol 10 ◽

pp. 140 ◽

Cited By ~ 1

Author(s):

José Fernando Guedes-Corrêa ◽

Rogério Pires Amorim ◽

Maristella Reis da Costa Pereira ◽

Rodrigo Salvador Vivas Cardoso ◽

Felipe D’Almeida Costa ◽

...

Keyword(s):

Brachial Plexus ◽

Malignant Neoplasm ◽

Cell Tumor ◽

Subtotal Resection ◽

Rapid Progression ◽

Adjuvant Radiation ◽

Round Cell ◽

Round Cell Tumor ◽

Small Round Cell Tumor ◽

Small Round Cell

Background: Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive malignant neoplasm typically located in the abdomen or pelvis. Other possible locations are the chest, pleura, scrotum, and central nervous system. DSRCT originally arising from the brachial plexus (BP) is extremely rare, to the best of our knowledge, only two cases have been previously described in the English scientific literature. Case Description: The authors present one new case of DSRCT arising from the left BP, the first in this location with rapid progression and in a female patient. We also highlight the importance of multimodal therapy, which included resection and both adjuvant radiation and chemotherapy. Macroscopic and microscopic characteristics of the lesion are detailed, as well as the patient’s status at 56-month follow-up. Conclusion: For primary BP DSRCT, aggressive subtotal resection followed by radiation and chemotherapy can be satisfactory for disease control and for maintaining or improving the neurological status.

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Desmoplastic small round cell tumor showing solid proliferation with limited desmoplasia and confusing immunohistochemical findings: an autopsy report

Medical Molecular Morphology ◽

10.1007/s00795-019-00242-5 ◽

2020 ◽

Vol 53 (3) ◽

pp. 177-182

Author(s):

Atsushi Kihara ◽

Kazuya Takahashi ◽

Ayataka Ishikawa ◽

Yusuke Amano ◽

Daisuke Matsubara ◽

...

Keyword(s):

Cell Tumor ◽

Round Cell ◽

Round Cell Tumor ◽

Small Round Cell Tumor ◽

Small Round Cell ◽

Autopsy Report

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FDG PET/CT imaging of desmoplastic small round cell tumor: findings at staging, during treatment and at follow-up

Pediatric Radiology ◽

10.1007/s00247-015-3315-y ◽

2015 ◽

Vol 45 (9) ◽

pp. 1308-1315 ◽

Cited By ~ 15

Author(s):

Austin Ostermeier ◽

M. Beth McCarville ◽

Fariba Navid ◽

Scott E. Snyder ◽

Barry L. Shulkin

Keyword(s):

Fdg Pet ◽

Ct Imaging ◽

Cell Tumor ◽

Round Cell ◽

Round Cell Tumor ◽

Small Round Cell Tumor ◽

Small Round Cell ◽

Pet Ct ◽

Fdg Pet Ct

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Desmoplastic Small Round Cell Tumor of the Submandibular Gland: A Case Report and Literature Review

ORL ◽

10.1159/000517563 ◽

2021 ◽

pp. 1-6

Author(s):

Qingjiao Li ◽

Xiaolu Yuan

Keyword(s):

Submandibular Gland ◽

Young Patients ◽

Neck Region ◽

Cell Tumor ◽

Pelvic Cavity ◽

Round Cell ◽

Round Cell Tumor ◽

Small Round Cell Tumor ◽

Small Round Cell ◽

The Right

Desmoplastic small round cell tumor (DSRCT) is a rare and aggressively malignant tumor mostly occurring in the abdominal and pelvic cavity of young patients. However, few cases had been reported concerning DSRCT occurring in the head and neck region. We presented a rare case of DSRCT of the right submandibular in a 25-year-old man. MRI revealed a 3 × 2-cm solid nodule located in the right submandibular, and physical examination showed no other occupying lesion elsewhere. Histologically, the tumor was composed of various-sized small round cell nests, embedded in an abundant desmoplastic stroma. Immunohistochemically, the tumor cells were typically positive for epithelial (CK and EMA), mesenchymal (vimentin and desmin), and neuroendocrine (CD56, NSE, Syn, and CgA) markers, but negative for WT1. Fluorescence in situ hybridization revealed the presence of a break apart involving the Ewing sarcoma (EWS) gene. The patient received chemotherapy and radiotherapy and relapsed after 19 months of follow-up. DSRCT of the submandibular gland is rare, and the diagnosis of this tumor in an uncommon location relies on the histomorphology, immunophenotype, and EWS gene translocation detection. Differential diagnosis including primary salivary gland tumors and the other small round cell tumors needs to be excluded.

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The Role and Clinical Effectiveness of Multiline Chemotherapy in Advanced Desmoplastic Small Round Cell Tumor

Clinical Medicine Insights Oncology ◽

10.1177/1179554920987107 ◽

2021 ◽

Vol 15 ◽

pp. 117955492098710

Author(s):

Hyehyun Jeong ◽

Yong Sang Hong ◽

Young-Hoon Kim ◽

Chan Wook Kim ◽

Si Yeol Song ◽

...

Keyword(s):

Medical Center ◽

Complete Resection ◽

Cell Tumor ◽

Line Treatment ◽

Round Cell ◽

First Line ◽

Round Cell Tumor ◽

Small Round Cell Tumor ◽

Small Round Cell ◽

First Line Treatment

Background: A multimodal approach is the standard treatment for desmoplastic small round cell tumor (DSRCT); however, many patients are diagnosed with inoperable disease, which leaves chemotherapy as the only treatment option. There are limited data on the effectiveness of palliative chemotherapy, especially when used after first-line treatment. Here, we evaluated the clinical outcomes of patients with DSRCT treated with multiple lines of chemotherapy. Methods: We reviewed medical records of 14 patients with pathologically confirmed DSRCT at Asan Medical Center between 2004 and 2018. Results: The median age at diagnosis was 25, with males comprising 92.9% of patients. All patients had inoperable disease at presentation and received chemotherapy as the initial treatment. Four patients (28.6%) were treated with surgery, and complete resection was achieved in 1 patient. Median overall survival (OS) was 23.9 months, and 1-, 2-, and 3-year survival rates were 92.9%, 48.6%, and 19.5%, respectively. In patients receiving first- (N = 14), second- (N = 10), and third-line (N = 8) chemotherapy, median time-to-progression was 9.9, 3.5, and 2.5 months, respectively, and the disease control rates were 100%, 88.9%, and 75.0%, respectively. Factors associated with longer OS in the univariable analysis were ⩽2 metastatic sites at presentation (27.0 vs 14.7 months; P = .024) and surgery with intended complete resection (43.5 vs 20.1 months; P = .027). Conclusions: Although advanced DSRCT may initially respond to chemotherapy after first-line treatment, the response becomes less durable as the disease progresses. Individualized treatment decisions focused on palliation should be made.

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