Malignant Rhabdoid Tumour of the Perineum in an Adult—A Rare Presentation

Singh, Rajdeep; Basra, Baljeet Kumar; Khurana, Nita; Sarda, A. K.

doi:10.1007/s12262-012-0657-7

Cited by 2 publications

(1 citation statement)

References 8 publications

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“…Classification of renal pelvic tumors as following: It includes four grade: Grade I includes papillary with normal mucosa (thickness and morphology), grade II includes papillary with minimal pleomorphisim, mitosis, giant cells, and invasion, grade III includes flat, transitional cells (plant life) to epidermoid (keratin forming) with significant pleomorphism, mitosis, giant cells and invasion, grade IV extreme pleomorphism or marked undifferentiation. Actinomycin D, vincristine, cisplatin, cyclophosphamide, adiramycin, etopside, [14] are among the chemotherapeutic drugs used to treat rhabdoid tumors, with no clear advantage of one over the other [15].…”

Section: Paediatricmentioning

confidence: 99%

A Case Report on Malignant Rhabdoid Round Cell Tumor in Pelvis

Kumutha

Baby

Sasikala

et al. 2021

JPRI

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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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Section: Paediatricmentioning

confidence: 99%

A Case Report on Malignant Rhabdoid Round Cell Tumor in Pelvis

Kumutha

Baby

Sasikala

et al. 2021

JPRI

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Embryonic signature distinguishes pediatric and adult rhabdoid tumors from other SMARCB1-deficient cancers

et al. 2017

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Extra-cranial rhabdoid tumors (RT) are highly aggressive malignancies of infancy, characterized by undifferentiated histological features and loss of SMARCB1 expression. The diagnosis is all the more challenging that other poorly differentiated cancers lose SMARCB1 expression, such as epithelioid sarcomas (ES), renal medullary carcinomas (RMC) or undifferentiated chordomas (UC). Moreover, late cases occurring in adults are now increasingly reported, raising the question of differential diagnoses and emphasizing nosological issues. To address this issue, we have analyzed the expression profiles of a training set of 32 SMARCB1-deficient tumors (SDT), with ascertained diagnosis of RT (n = 16, all < 5 years of age), ES (n = 8, all > 10 years of age), UC (n = 3) and RMC (n = 5). As compared with other SDT, RT are characterized by an embryonic signature, and up-regulation of key-actors of de novo DNA methylation processes. Using this signature, we then analysed the expression profiling of 37 SDT to infer the appropriate diagnosis. Thirteen adult onset tumors showed strong similarity with pediatric RT, in spite of older age; by exome sequencing, these tumors also showed genomic features indistinguishable from pediatric RT. In contrary, 8 tumors were reclassified within carcinoma, ES or UC categories, while the remaining could not be related to any of those entities. Our results demonstrate that embryonic signature is shared by all RT, whatever the age at diagnosis; they also illustrate that many adult-onset SDT of ambiguous histological diagnosis are clearly different from RT. Finally, our study paves the way for the routine use of expression-based signatures to give accurate diagnosis of SDT.

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Malignant Rhabdoid Tumour of the Perineum in an Adult—A Rare Presentation

Cited by 2 publications

References 8 publications

A Case Report on Malignant Rhabdoid Round Cell Tumor in Pelvis

A Case Report on Malignant Rhabdoid Round Cell Tumor in Pelvis

Embryonic signature distinguishes pediatric and adult rhabdoid tumors from other SMARCB1-deficient cancers

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