Gastrointestinal Stromal Tumour

          

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      Atypical Fibroxanthoma

            Dr Sampurna Roy MD

 
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  May 2007

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CONGENITAL SMOOTH MUSCLE HAMARTOMA

PILOLEIOMYOMA

ANGIOLEIOMYOMA

LIPOLEIOMYOMA

GENITAL LEIOMYOMA

LEIOMYOSARCOMA

RHABDOMYOMATOUS MESENCHYMAL HAMARTOMA

RHABDOMYOMA

RHABDOMYOSARCOMA

               

Rhabdomyomatous mesenchymal hamartoma is a rare congenital lesion of the dermis and soft tissues.

This entity exists under various names including striated muscle hamartoma, congenital midline hamartoma, and hamartoma of cutaneous adnexa and mesenchyme.

Age: Usually presents in neonates or infants. Cases have been reported in adults.

Site: Presents as one or more small cutaneous polyps in the head and neck region.

Microscopic features:  The lesion consists of a disordered and varied collection of mature adipose tissue, skeletal muscle, adnexal elements and nerve bundles. Bundles of skeletal muscle is present in the reticular dermis which may extend into the subcutis.

This lesion may be associated with other congenital anomalies and anomaly syndromes hence systemic evaluation of patients diagnosed with this entity has been recommended.

                      

 
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Abstracts:

Rhabdomyomatous mesenchymal hamartoma: a plaque-type variant in an adult. Kaohsiung J Med Sci. 2005;21(4):185-8.

Rhabdomyomatous mesenchymal hamartoma associated with nasofrontal meningocele and dermoid cyst. J Cutan Pathol. 2005;32(4):310-3.

Rhabdomyomatous mesenchymal hamartoma: an unusual dermal entity with a report of two cases and a review of the literature. J Cutan Pathol. 2002;29 (4):238-43.

Rhabdomyomatous mesenchymal hamartoma of the eyelid: report of a case and literature review.Ophthalmology. 2001 ;108(4):798-804.

Rhabdomyomatous mesenchymal hamartoma. Am J Dermatopathol. 1994;16 (1) : 73-5.

Rhabdomyomatous mesenchymal hamartoma of skin. Pediatr Pathol. 1992;12 (5):731-5.

Multiple rhabdomyomatous mesenchymal hamartomas of skin. Am J  Dermato pathol. 1990;12(5):485-91.

Congenital midline hamartoma: case report with histochemical and immuno-histochemical findings. Pediatr Dermatol. 1990 Sep;7(3):199-201.

Rhabdomyomatous mesenchymal hamartoma of skin.J Dermatol. 1990; 17(6): 384-7.

Rhabdomyomatous mesenchymal hamartoma of skin. Am J Dermatopathol. 1989 ;11(1):58-63.
Soft Tissue Pathology;

Myxoid Tumours of Soft Tissue Classification of Soft Tissue Tumour;  Gross examination of soft tissue specimen ;  A practical approach to histopathological reporting of soft tissue tumours Grading of soft tissue tumours ; Lipomatous tumours ;Neural tumours ; Myogenic tumours ;Vascular tumours ;Fibroblastic/Myofibroblastic tumours ; Myofibroblastic tumours ;  Fibrohistiocytic tumours ; ChondroOsseous tumours ; Soft TissueTumours of Uncertain Differentiation ; Notochordal Tumour -Chordoma ;Extra-adrenal Paraganglioma ; Gastrointestinal Stromal Tumour ;