http://www.histopathology-india.net/SoftTissuePath.htm

Malignant rhabdoid tumour is a rare soft-tissue neoplasm that occurs in children and young adults. 

Given their highly aggressive nature, early diagnosis of rhabdoid tumours is essential. Death usually occurs within 2 years. Metastases occur early to lungs, liver & lymphnodes.

Site:   It was first described as as renal tumour, subsequently the tumour was found in soft tissue and in other sites . Cases have involved numerous extrarenal sites, including the thymus, liver, paravertebral region, central nervous system, heart, prostate, pelvis, chest wall, extremities, as well as soft tissues.

Age:  Most patients are infants or children.

Microscopic features:

1. Round or epitheloid cells with hyaline globular cytoplasmic inclusions and very large nucleoli within vesicular nuclei.

2. Deep & homogeneous acidophilic cytoplasm of the tumor cells is characteristic.

3. Myxoid, pseudoalveolar & hyalinized areas may be seen.

'Rhabdoid inclusions' are present in epithelioid sarcoma, synovial sarcoma, intra-abdominal desmoplastic small cell tumour, rhabdomyosarcoma,  melanoma & various types of carcinoma.

Immunohistochemistry:   Consistent positivity for cytokeratin, EMA & vimentin.  S-100 protein is usually negative.

Electron microscopy:    Inclusions are paranuclear aggregates of intermediate filaments. Image

Visit:RHABDOMYOMATOUS MESENCHYMAL HAMARTOMA ; 

         RHABDOMYOMA ; CARDIAC RHABDOMYOMA

Abstracts:

Primary rhabdoid tumor of the brain in an adult.Neuropathology. 2006 Feb;26(1):57-61.

Urothelial carcinoma with rhabdoid features: report of 6 cases.Hum Pathol. 2006 Feb;37(2):168-72.

Extrarenal malignant rhabdoid tumour of the heel--a case report. Anticancer Res. 2005 Nov-Dec;25(6C):4573-6.

Cutaneous extrarenal rhabdoid tumor with myogenic differentiation.J Cutan Pathol. 2005 Nov;32(10):690-5

 "Proximal type" epithelioid sarcoma of the vulva: differential diagnosis with other extrarenal rhabdoid tumors.Pathologica. 2005 Jun;97(3):133-6.

Skeletal muscle-like and rhabdoid cells in uterine leiomyomas.Int J Gynecol Pathol. 2005 Oct;24(4):319-25.

Congenital orbital and disseminated extrarenal malignant rhabdoid tumor.Ophthal Plast Reconstr Surg. 2005 Jan;21(1):76-9.

Soft-tissue extrarenal rhabdoid tumor with a unique long-term survival.Ultrastruct Pathol. 2004 Jan-Feb;28(1):49-52.

Pathologic quiz case. Extrarenal rhabdoid tumor.Arch Pathol Lab Med. 1999 Sep;123(9):853-4.

Extrarenal rhabdoid tumors of soft tissue: a clinicopathologic and immunohistochemical study of 18 cases.Ann Diagn Pathol. 1998 Dec;2(6):351-62.

"Proximal-type" epithelioid sarcoma, a distinctive aggressive neoplasm showing rhabdoid features. Clinicopathologic, immunohistochemical, and ultrastructural study of a series.Am J Surg Pathol. 1997 ;21(2):130-46.

Malignant rhabdoid tumors: a clinicopathologic review and conceptual discussion. Semin Diagn Pathol 1995;12:233–248.  

The clinicopathologic spectrum of putative extrarenal rhabdoid tumors: an analysis of 42 cases studied with immunohistochemistry or electron microscopy. Am J Surg Pathol 1994;18:1010–1029.

Primary malignant rhabdoid tumor of the spinal dura. Clin Neuropathol 1994;13:221–224.

Paravertebral malignant rhabdoid tumor in an adult. A case report with immunocytochemical study.Pathol Res Pract. 1987 Oct;182(5):713-8.

Renal and extrarenal rhabdoid tumors in children: a clinicopathologic study of 14 patients.Semin Diagn Pathol. 1986 May;3(2):151-63.