Gastrointestinal Stromal Tumour

          

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

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            Dr Sampurna Roy MD

Diagnosis of Path Case19: Granular cell tumour
Diagnosis of Path Case10:Congenital Gingival Granular Cell Tumour (Congenital Epulis)

           Case19: Case history and images:

           Case 10: Case history and images:

 
July 2008 
       Gastrointestinal Stromal Tumour

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

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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

Fibroblastic/Myofibroblastic tumours

Myofibroblastic tumours

Fibrohistiocytic tumours

ChondroOsseous tumours

Soft TissueTumours of Uncertain Differentiation               

Notochordal Tumour - Chordoma

Extra-adrenal Paraganglioma

Gastrointestinal Stromal Tumour

Reactive and hamartomatous lesions:

Traumatic neuroma

Morton's neuroma

Digital Pacinian neuroma

Nerve Sheath Ganglion

Fibrolipomatous hamartoma of nerve

Benign tumours:

Solitary circumscribed neuroma
(palisaded encapsulated neuroma)

Schwannoma (neurilemmoma)
and variants                          

Neurofibroma and variants 

Perineurioma   

Dermal nerve sheath myxoma
(neurothekeoma)

Cellular neurothekeoma

Granular cell tumour

              
Malignant tumour:

Malignant peripheral nerve sheath tumour
  


Neuroendocrine Carcinoma:

Merkel cell (neuroendocrine) carcinoma

Malignant primitive neuroectodermal tumour   

Miscellaneous neuroectodermal tumours presenting in soft tissue

Subcutaneous Myxopapillary Ependymoma  

Heterotopic Glial Nodule

Heterotopic Meningeal Lesions

Intramuscular Myxoma

Aggressive Angiomyxoma

Ossifying fibromyxoid tumour

Synovial Sarcoma

Alveolar Soft Part Sarcoma

Epithelioid Sarcoma

Clear cell sarcoma & PEComas

Desmoplastic small round cell tumour

Pleomorphic hyalinizing angiectatic tumour

Mixed tumour, myoepithelioma, parachordoma

Ectopic hamartomatous thymoma

Extra-renal rhabdoid tumour

Malignant mesenchymoma

                  
Granular cell tumour is a benign tumour of disputed histogenesis. Some authors support a Schwann cell origin.

Age: Usually occurs in middle aged adults (40-50 years)

Site: It is most common in the tongue, followed by the anterior and posterior chest wall and the upper limbs. The tumour also occur on the head and neck region and in the larynx, stomach, vulva & anogenital region.
             Visit: Granular Cell Tumour of Bronchus

Clinical presentation:
The lesion is common in blacks  and presents as a solitary  yellow-tan nodule in the dermis or subcutis and less frequently in the submucosa, smooth muscle or striated muscle.

Microscopical features: Image 1 ; Image2 Image3 Image4.
Granular cell tumour is a nonencapsulated tumour  with a  poorly defined  margin.
The tumour is  arranged in large sheets or in nests and may extend into the subcutis.
It is composed of large polygonal cells with coarse eosinophilic cytoplasm and central hyperchromatic nucleus and occasional eosinophilic droplets.
The cells are PAS positive and diastase resistant.
Nests of tumour cells may surround dermal appendages and may be present within the epineurium of nerve fibres.
Elastosis is common in the backround stroma. 
Some cases are associated with marked acanthosis or pseudoepitheliomatous hyperplasia of the overlying squamous epithelium.

Immunohistochemistry:  The tumour cells show  positivity with S100 protein, neuron-specific enolase, vimentin, PGP9.5  and melanoma-associated antigen (NK1/C-3). The pan-macrophage marker, CD68 (Kp1) reacts strongly with granular cell tumour.

Features indicating malignancy:   Tumour size more than 5  cms, presence of vascular invasion, necrosis and rapid growth. Other features include brisk mitotic activity(>2/10 high-power fields)., pleomorphism, presence of spindling of cells and angiogenesis. Three or more of these features are required for a malignant diagnosis.  If fewer than 3 are present, the tumour is considered to be “atypical granular cell tumor” and typically has a good prognosis if fully excised. If metastatic lesions are found in bone, lymph nodes, peripheral nerves, peritoneal cavity or lungs - the prognosis is usually very poor.

Variants:

Gingival granular cell tumour of newborn (congenital epulis): Congenital gingival granular cell tumours are rare benign tumours of uncertain histogenesis.

Site: Alveolar ridge of the maxilla in newborns . Sex: Striking predilection for female infants  suggests the presence of an endogenous hormonal stimulus in utero.

Microscopic features: Image 1 ; Image2 Image3 Image4

Like adult granular cell tumour  this lesion is composed of polygonal cells with abundant granular cytoplasm and small eccentric nuclei with occasional small nucleoli. There is a prominent vascular stroma with perivascular lymphocytes and histiocytes. Entrapped non-neoplastic odontogenic epithelium may be present in some cases. There is no pseudoepitheliomatous hyperplasia of the overlying squamous mucosa and no nerve bundles are seen within these lesions.

The tumour is negative when immunostained with S100 protein, in contrast to adult granular cell tumours.

Differential diagnosis: Teratoma, leiomyoma, congenital dermoid cyst,  congenital fibrosarcoma, and congenital lipoma.


Polypoid granular cell tumour: The tumours are composed of elongated spindle-shaped to polygonal or round cells with prominent granular cell change. The tumour nuclei may show mild focal atypia to moderate atypia. There is brisk mitotic activity .These tumours do not show any specific differentiation. 

                    

                 (ESCOP)IMAGE LINKS: 1  2  3  4  5  (Dr Weems)

Abstracts:

Primitive nonneural granular cell tumors of skin: clinicopathologic analysis of 13 cases. Am J Surg Pathol. 2005 Jul;29(7):927-34

Dermal non-neural granular cell tumour (so-called primitive polypoid granular cell tumour): a distinctive entity further delineated in a clinicopathological study of 11 cases.Histopathology. 2005 Aug;47(2):179-85.

A rare case of multiple congenital epulis.J Matern Fetal Neonatal Med. 2004 Nov;16 Suppl 2:55-8

Malignant granular cell tumor: a clinicopathologic analysis of 10 cases with review of literature.Zhonghua Bing Li Xue Za Zhi. 2004; 33(6):497-502

Congenital epulis: a rare diagnosis in paediatric surgery.Eur J Pediatr Surg. 2002 Apr;12(2):124-6

Multiple congenital epulis of the alveolar ridge and tongue.Ann Plast Surg. 2001 Aug;47(2):199-202

Malignant granular cell tumor of soft tissues: a study of two new cases. Int J Surg Pathol. 2001 Jul;9(3):255-9

Congenital Epulis. PEDIATRICS Vol. 107 No. 2 February 2001, p. e22 (FULL TEXT)

Cellular heterogeneity of granular cell tumours: a clue to their nature?J Oral Pathol Med. 2000 Jul;29(6):284-90.

Infiltrating granular cell tumor of the esophagus: a description of two cases.Ann Pathol. 2000 Mar;20(2):158-62

Malignant granular cell tumor of soft tissue: diagnostic criteria and clinico pathologic correlation. Am J Surg Pathol. 1998 Jul;22(7):779-94.

Granular cell tumors.Ann Otol Rhinol Laryngol. 1994 May;103(5 Pt 1):416-9.

Immunoreactivity of S100 protein, alpha-1-antitrypsin, and CD68 in adult and congenital granular cell tumors.Mod Pathol. 1996 Sep;9(9):888-92

Expression of calretinin and the alpha-subunit of inhibin in granular cell tumors.Am J Clin Pathol. 2003 Feb;119(2):259-64

Expression of PGP 9.5 in granular cell nerve sheath tumors: an immunohistochemical study of six cases. J Cutan Pathol. 2001 Jul;28(6):282-6

Granular cell tumor: immunohistochemical assessment of inhibin-alpha, protein gene product 9.5, S100 protein, CD68, and Ki-67 proliferative index with clinical correlation. Arch Pathol Lab Med. 2004 Jul;128(7):771-5

Dermatofibroma-like granular cell tumor.J Cutan Pathol.2001;28(1):49-52.

Immunoreactivity in granular cell tumours of the larynx.Auris Nasus Larynx. 1999 Jul;26(3):305-10

Granular cell tumors of the esophagus: a clinical and pathologic study of 13 cases.Ann Thorac Surg. 1996 ;62(3):860-5

Congenital epulis and granular cell tumor: a histologic and immunohistochemical study.Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1995 Dec;80(6):687-97.

CD68 and factor XIIIa expressions in granular-cell tumor of the skin.Dermatology. 1993;186(2):106-8

Primitive polypoid granular-cell tumor and other cutaneous granular-cell neoplasms of apparent nonneural origin.Am J Surg Pathol. 1991 Jan;15(1):48-58

Congenital granular cell tumor with systemic involvement. Immunohistochemical and ultrastructural study.Arch Pathol Lab Med. 1991 S;115(9):934-8

Oral granular cell tumors: a clinicopathologic and immunocytochemical study.Oral Surg Oral Med Oral Pathol. 1988 Apr;65(4):427-35

Gingival granular cell tumors of the newborn (congenital "epulis"): a clinical and pathologic study of 21 patients.Am J Surg Pathol. 1981 Jan;5(1):37-46

 

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