Soft-Tissue Extrarenal Rhabdoid Tumor with a Unique Long-Term Survival

Abstract

Rhabdoid tumors of kidney and extrarenal rhabdoid tumors are identified by a round-epithelioid cell morphology and a bland immunophenotype, but a distinctive ultrastructure dominated by paranuclear whorls of intermediate filaments, most usually of vimentin. These tumors are also known to be highly aggressive malignancies, which, typically, bear a poor prognosis, frequently measured in months following initial presentation. The authors record the case a soft-tissue rhabdoid tumor in a 12-year-old boy with a unique long-term survival in excess of 16 years. The features of this case are documented, with a brief summary of histological, immunohistochemical, ultrastructural, and genetic characteristics of this entity.

A rhabdoid‐mimicking intrahepatic cholangiocarcinoma

“…2,3 Although extra-renal rhabdoid tumors (ERTs) arising in the livers of pediatric patients (hepatic rhabdoid tumors; HRTs) have a poor outcome, 4 there have been some recent reports of exceptional ERTs affecting young adults or showing outcomes that were not necessarily poor, but with pathological features that varied among the cases. [3][4][5][6] Here we present a case of rhabdoidmimicking IC and discuss the differences between this case and conventional ERTs on the basis of immunohistochemical and ultrastructural observations.…”

“…5 Ultrastructurally, RT cells show coexpresson of vimentin and low-molecularweight cytokeratin with characteristic whorls of intermediate filaments. 7 Although many ERTs with exceptional clinical features have been reported, i.e., those affecting adults 4 and not necessarily showing a poor outcome, 6 they may not show the same immunohistochemical phenotype, or have not been studied ultrastructurally. [3][4][5][6] The present case simulated ERT morphologically but was negative for vimentin with looser aggregates of intermediate filaments, and had a favorable outcome.…”

“…7 Although many ERTs with exceptional clinical features have been reported, i.e., those affecting adults 4 and not necessarily showing a poor outcome, 6 they may not show the same immunohistochemical phenotype, or have not been studied ultrastructurally. [3][4][5][6] The present case simulated ERT morphologically but was negative for vimentin with looser aggregates of intermediate filaments, and had a favorable outcome. The subcellular coexpression of vimentin and keratin has been reported to be diffuse for the former and showing a paranuclear globular pattern for the latter, 7 as was seen in some of the cells in the present case.…”

Congenital disseminated malignant rhabdoid tumor of the soft tissue

“…We came across to 32 patients with extrarenal MRT cases under 3 years old and only few of those were congenital 1–3,5–7. The treatment of disseminated MRT is very difficult 3–5,8,9. Kodet et al 3 reported that almost all patients less than 1 year of age died within months despite multi‐agent chemotherapy in all patients (n:11) plus radiation therapy in three patients.…”

Extrarenal rhabdoid tumor: A review of literature and a report of cases with atypical morphology

“…Опухоль встречается во всех возрастных категориях от 0 до 18 лет с медианой 8-9 лет [1,16]. Описаны случаи врожденных ЭРО [1,17] и редкие случаи возникновения опухолей у взрослых пациентов [18][19][20][21]. Мальчики болеют незначительно чаще девочек в соотношении 1,5:1 с частотой встречаемости 15 случаев в год у детей в возрасте до 15 лет в Северной Америке, 6 случаев в год в Германии [1,8,9].…”