Gastrointestinal Stromal Tumour

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 SMALL INTESTINE

 LARGE INTESTINE

 

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        Dr Sampurna Roy MD

 
 August 2009
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B-CELL LYMPHOMA :  

- MALTOMA :  Gastric Lymphoma:  click
-IMMUNOPROLIFERATIVE SMALL INTESTINAL DISEASE
-BURKITT'S LYMPHOMA
-MANTLE CELL LYMPHOMA
-FOLLICULAR LYMPHOMA
-PLASMACYTOMA
-OTHER  B-CELL LYMPHOMAS (mostly high grade)

 

T- CELL LYMPHOMA:

-CELIAC ENTEROPATHY ASSOCIATED : click
-T-CELL LYMPHOMA WITH TISSUE EOSINOPHILIA
-OTHER T-CEL LYMPHOMA

Gastrointestinal tract is the most common site of primary extranodal lymphoma . Lymphoma accounts for 30% of small bowel malignancies.

Primary gastrointestinal lymphoma exhibits no evidence of liver, spleen or bone marrow involvement at the time of diagnosis. Regional  lymphnode involvement may be present.

Criteria used to classify malignant lymphoma of small bowel include:
i) Cell lineage: B or T cell lymphoma

ii) Primary or systemic nature of tumour

iii)Associated  backround chronic illnesses.

Primary gastrointestinal lymphoma usually arise as a sporadic tumour.

Sporadic lymphomas, also termed the 'Westerm type'  are B- cell lymphomas which appear to arise from the B- cells of the mucosa associated lymphoid tissue.

Some lymphomas develop in the backround  of chronic  diseases . Eg.  Sprue -like malabsorption syndromes, congenital immunodeficiency states, infection with HIV following organ transplantation with immunosuppresion (heart transplant recipients), those complicating Crohn's disease , hypogammaglobulinaemia with lymphoid hyperplasia, in patients with alpha chain disease.

Ileum is commonly affected followed by jejunum and duodenum.

Immunoproliferative small intestinal disease, / Mediterranean lymphoma:

B-cell lymphoma usually arises in children and young adults with Mediterranean ancestry.
Usually located in the distal portion of duodenum and upper jejunum.
Diffusely thickened bowel wall with small nodules or discrete tumour masses indicate high grade tumour.
Diagnosis can be made by endoscopic biopsy. Histologically the low grade tumour is characterized by an infiltrate of plasma cells and lympho-plasmacytoid cells in the mucosa. There is broadening & shortening of villi and separation of crypts. The cells appear mature. In the early stage the tumour responds to antibiotics.
High grade tumour is characterized by pleomorphic large cells with immunoblastic and plasmacellular features.
Immunohistochemistry reveals alpha heavy chains of immunoglobulins in the cytoplasm.

Burkitt's lymphoma of small bowel:

-Relatively common in the Middle east and Algeria.
-Usually located in the ileum and the ileocaecal region.
-May cause intussusception.
-Histologically, the tumour consists of lymphoblast like cells with high mitotic rate
interspersed with macrophage containing cellular debris (starry-sky). The infiltrating tumour  dissects through the muscle fibres and surrounds the regional lymphnodes without infiltrating them.
-Immunohistochemistry:  CD19,20,22 &79a : positive
CD 10: positive,
CD5 & 23:  negative
Ki67 : more than 85% cell are positive.

Mantle cell lymphoma of small bowel:

Presents as lymphoid polyps in elderly patients (around 60 years of age).
It is usually associated with leukemic spread.
Immunohistochemistry:  Cyclin D1 positive
CD5 positive
CD10 : negative / positive
CD19,20,22, 79a  positive
CD23 : negative

Follicular lymphoma of the small bowel:

The tumour has a follicular growth pattern and consists
of a mixture of germinal
centre blasts and cleaved
cells ( centroblasts and centrocytes).
Immunohistochemistry:  CD5 : negative
CD10 : positive/negative
CD19, 20, 22, 79a : positive
BCL-2 : positive

Occasionally, these  tumours transform into diffuse tumour containing large centroblasts. (Diffuse large B- cell lymphoma).

ABSTRACTS:

-Critical evaluation of Bcl-6 protein expression in diffuse large B- cell lymphoma of the stomach and small intestine. Am J Surg Pathol. 2003;27(6):790-8

Primary follicular lymphoma of the gastrointestinal tract: a study of 25 cases and a literature review.  Ann Oncol 2003 ; 14(4): 623-9 

Primary follicular lymphoma of the gastrointestinal: a clinical and pathologic study of 26 cases. Am J Surg Pathol. 2002; 26(2) : 216-24

Problems in biopsy differential diagnosis in lymphomas of the small and large intestine. Verh Dtsch Ges Pathol 1999; 83: 90-100

Classification of intestinal T- cell neoplasms and their differential diagnosis. Am J Clin Pathol.1999; 111: (1 Suppl 1): S68-74

Primary lymphoma of the small intestine. A clinico-pathologic study of 119 cases. Am J Surg Pathol.1993, 17: 429-442

Gastrointestinal lymphomas of T and B cell types.  Mod Pathol.1999; 12(2): 151- 8

Extranodal lymphomas: the MALT concept. Verh Dtsch Ges Pathol.1992 ;76:14-23 abstract

                     

- Lymphoid hyperplasia of small intestine is a reactive process.

Path Quiz Case51

- Usually located at the ileocecal junction.

- Clinically the patients present with right iliac fossa pain, obstruction and sometimes  intussusception.

- Associated with viral infection in children.

- Diffuse nodular lymphoid hyperplasia is present in some patients of primary hypogammaglobulinemia.

- Giardia lamblia infection is noted in some patients with lymphoid hyperplasia.

- D/D:  Malignant lymphoma: Immunohistochemistry is performed to confirm the diagnosis

ABSTRACTS

Lymphoid hyperplasia of the intestine in children. 15 cases. Ann Pediatr (Paris)1992; 39(6): 359-64

Florid reactive lymphoid hyperplasia of the terminal ileum in adults. A condition bearing a close resemblance to low-grade malignant lymphoma. Histopathology 1990;17: 19-26

Nodular lymphoid hyperplasia of the intestine tract in infancy and childhood. J Pediatr Surg.1985; 20: 25-29

Nodular lymphoid hyperplasia of the bowel in primary hypogammaglobulinaemia: a study of in vivo and in vitro lymphocyte function Gut 1977;18: 364-372

                 

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 IMAGES

Immunoproliferative small intestinal disease


Mantle cell lymphoma


Follicular lymphoma of
terminal  ileum


Burkitt's lymphoma
of small intestine


Lymphoid hyperplasia of small bowel 
(pseudolymphoma)


Normal histology of the small intestine for anatomic pathologists

An approach to evaluation of small intestinal biopsy.

Malabsorption syndrome (causes  and clinical investigations)

Tropical Sprue

Coeliac Disease

Enteropathy-associated T-cell lymphoma

Intestinal lymphangiectasia

Pathogens commonly affecting Small Intestine

Ascariasis

Cryptosporidium

Cytomegalovirus infection

Giardiasis

Hookworm Infection

Isosporiasis

Microsporidia

Mycobacterium Avium Intracellulare

Schistosomiasis

Whipple's disease

Lesions causing small bowel obstruction and bleeding - 
Intussusception : Adhesions : Volvulus

Meckel's diverticulum

Ischemic bowel disease 

Brunner's Gland Adenoma

Duodenal  Gangliocytic Paraganglioma

Lymphoma of the small intestine

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

Vascular 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


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