Gastrointestinal Stromal Tumour


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               Dr Sampurna Roy MD

August 2009


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Adnexal Tumours of the eyelid

Mesenchymal Tumour of the Eyelid

Pulmonary Mesenchymal Tumours

Cardiac fibroma

Nodular fasciitis

Proliferative fasciitis

Proliferative myositis

Ischaemic fasciitis


Fibrous Hamartoma of Infancy

Infantile Myofibromatosis/ Myofibroma

Juvenile hyaline fibromatosis

Inclusion  Body Fibromatosis

Calcifying aponeurotic fibroma

Fibromatosis colli

Fibroma of tendon sheath

Desmoplastic fibroblastoma (collagenous fibroma)

Storiform Collagenoma (sclerotic fibroma)

Giant Cell Collagenoma

Pleomorphic Fibroma



Cellular Angiofibroma

Giant Cell Angiofibroma



Solitary fibrous tumour

[Hemangiopericytoma  including Lipomatous Hemangiopericytoma]

Inflammatory myofibroblastic tumour

Low grade myofibroblastic sarcoma

Myxoinflammatory fibroblastic sarcoma

Infantile fibrosarcoma

Adult fibrosarcoma


Low grade fibromyxoid sarcoma

Hyalinizing Spindle Cell Tumour with Giant Rosettes

Sclerosing epithelioid fibrosarcoma

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


Infantile Myofibromatosis (IMF) was first described by Stout in 1954 as 'congenital generalized fibromatosis' and was renamed as infantile myofibromatosis by Chung and Enzinger in 1981 after recognition of the myofibroblastic nature of the lesion.

(1)Solitary myofibromatosis: Characterized by the presence of one nodule in the skin, muscle, bone or subcutaneous tissue; and 

(2)Multicentric type which can be divided into two sub-types :(2a)Multicentric lesions but without visceral involvement. (2b)Visceral involvement is present.

(3) Adult-type myofibroma

Note: The term myopericytoma was first proposed in 1996 by Requena as an alternative designation for ' solitary myofibroma' . It was later adopted in 1998 to describe a spectrum of tumours with striking concentric perivascular proliferation of spindle cells (perivascular myoid cells). [This spectrum includes - infantile-type myofibromatosis ; solitary myofibroma ; benign myopericytoma; infantile hemangiopericytoma ; glomangiopericytoma]

IMF is the most common fibrous tumour of infancy and must be considered when evaluating children who present with either solitary or multiple tumours, particularly during the neonatal period. 

In Solitary myofibromatosis  skin, subcutaneous tissue and skeletal muscle are affected most frequently, usually in the head, neck and trunk.  The most frequently affected bones are the skull, vertebrae, ribs, femur and tibia. Solitary lesions involving the viscera are rare.  Solitary or multicentric lesions confined to skin, soft tissues or bone has a good prognosis. These are usually cured by simple excision.

In the generalized form, the most common locations are the lung, heart, gastrointestinal tract and pancreas, as well as rarely the central nervous system., Infants with generalized visceral lesions have the worst prognosis.  Death in these cases often occurs due to cardio-pulmonary or gastrointestinal complications.

Solitary Myofibroma - Adult lesions are often solitary and superficial in nature. The tumour usually presents as a cutaneous nodule in head and neck region. Visceral and osseous lesions have not been reported.

Gross features:  The lesion may present as a firm scar or a superficially located well circumscribed but uncapsulated nodule. The deeper lesions are more infiltrative and diffuse in nature.

Histopathological features: 

Infantile Myofibromatosis:(ESCOP)


Deep seated myofibromatosis:(ESCOP)


Adult Myofibroma: (Dr Weems) IMAGE1  



Microscopically, each nodule has a central and peripheral area.

The peripheral area consists of spindle cells (myofibroblasts) with eosinophilic cytoplasm and ovoid nuclei arranged in well-demarcated short bundles and fascicles resembling smooth muscle.

The central portion consists of less differentiated rounder cells with pale cytoplasm and basophilic, small round nuclei, arranged in a hemangiopericytoma-like pattern.

Hemangiopericytomatous and myofibroblastic components occur in variable proportions.

More primitive areas show necrosis. Focal areas of calcification may be present.  Mild to moderate nuclear pleomorphism can be found in most lesions. Normal mitotic figures may be quite common. Abnormal mitotic figures are usually not present.

Vascular invasion (subendothelial proliferation of perivascular spindle cells) is common, but does not affect the prognosis.


Cells are vimentin and alpha-smooth muscle actin - Positive.   Desmin- shows inconstant expression.  S100 protein, epithelial membrane antigen & keratin - Negative


Differential Diagnosis:  Nodular fasciitis ;  NeurofibromaSchwannoma ;low-grade MPNST;  Dermatofibroma ; Leiomyoma ; Fibromatosis; Juvenile hyaline fibromatosis ; fibroma of tendon sheath ;

Infantile hemangiopericytoma- Infantile hemangiopericytoma versus infantile myofibromatosis. Study of a series suggesting a continuous spectrum of infantile myofibroblastic lesions. Am J Surg Pathol 1994 Sep;18(9):922-30

Inflammatory myofibroblastic tumour ;  Low grade myofibrosarcoma ;

Sections from the central cellular portion may show features mimicking small round cell sarcomas with a hemangiopericytoma-like vasculature: Eg-  PNET , mesenchymal chondrosarcoma, poorly differentiated synovial sarcoma


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Solitary cutaneous infantile myofibromatosis. Ann Pathol. 2004;24(5):427-31

Infantile myofibromatosis of the central nervous system. Childs Nerv Syst. 2003

Infantile myofibroma in a prematurely born twin: a case report.Pediatr Dermatol. 2003;20(4):345-9.

Orbital and periorbital myofibromas in childhood: two case reports. Ophthalmology. 2003;110(10):2000-5.

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Adult acral cutaneous myofibromas in a patient with generalized morphea. J Am Acad Dermatol. 2002; 46(6):953-6

Infantile myofibromatosis with visceral involvement and complete spontaneous regression.J Dermatol. 2001;28(7):379-82

Systemic myofibromatosis in an infant. An Esp Pediatr. 2001;55(1):83-6.

Myofibromas and myofibromatosis of the oral region: A clinicopathologic analysis of 79 cases.Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2000;89(1):57-65.

Myofibromas presenting in the oral cavity: a series of 9 cases.Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2000 Mar;89(3):343-8.

Solitary form of infantile myofibromatosis: a histologic, immunohistochemical, and electronmicroscopic study of a regressing tumor over a 20-month period.Am J Dermatopathol 1999 Aug;21(4):375-80

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Myofibromatosis in adults, glomangiopericytoma, and myopericytoma: a spectrum of tumors showing perivascular myoid differentiation. Am J Surg Pathol 1998 May;22(5):513-25

Cutaneous adult myofibroma: a vascular neoplasm.J Cutan Pathol. 1996 Oct;23(5) :445-57

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Facial myofibroma of the newborn simulating sarcoma.Ann Dermatol Venereol. 1996;123(9):577-80

Congenital generalized myofibromatosis: a disseminated angiocentric myofibromatosis.Pediatr Pathol Lab Med 1995;15(4):571-87

Monophasic cellular variant of infantile myofibromatosis. An unusual histopathologic pattern in two siblings.  Am J Dermatopathol 1995;17(2):131-8

Massive apoptosis in infantile myofibromatosis. A putative mechanism of tumor regression.Am J Pathol 1994;144(3):480-5

Solitary myofibroma in adults: clinicopathological analysis of a series. Histopathology.1993;22(4):335-41.

Familial occurrence of infantile myofibromatosis. Cancer 1992;69(5): 1294-9

Cutaneous myofibroma . Mod Pathol.1989;2(6):603-9.

Infantile myofibromatosis: the most common fibrous tumor of infancy. J Pediatr Surg 1988;23:315-8.

Infantile myofibromatosis: the most common fibrous tumor of infancy. J Pediatr Surg 1988 Apr;23(4):315-8

Infantile myofibromatosis. Evidence for an autosomal-dominant disorder. Am J Surg Pathol 1984;8:529-38.

Infantile myofibromatosis.Cancer. 1981 Oct 15;48(8):1807-18.

Solitary congenital fibromatosis (infantile myofibromatosis) of bone. Am J Surg  Pathol. 1991;15(10): 935-41.

Infantile myofibromatosis: report of two cases. J Dermatol.1994;21(7):508-13.

Congenital hemangio- pericytoma /infantile myofibro matosis: radical surgery versus a conservative "wait and see" approach.Pediatr Hematol Oncol 1997; 14(4):387-93

New entities, concepts, and questions in childhood tumor pathology.Gen Diagn Pathol.1995;141(1):1-14.

Acquired benign myofibroma of the skin (adult myofibroma) Hautarzt. 1993 ;44 (9):561-8.


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