Carinoid tumour of the ileum with metastasis to orbit

Case contributed by RMH Neuropathology

Presentation

Orbital nodule stable in size for 18 months. Later was found to have the ileal tumour

Patient Data

Age: 63
Gender: Female

A well circumscribed right orbital mass is noted demonstrating homogeneous contrast enhancement. It displaces the medial rectus inferiorly and the superior rectus laterally. The optic nerve is displaced inferolaterally. 

Pathology

Histology (orbit)

MACROSCOPIC DESCRIPTION:

An irregular grey tan nodule, 20x10x10mm. Inked blue and serially sectioned.

MICROSCOPIC DESCRIPTION:

Paraffin sections show a densely hypercellular tumour. This consists of cells with uniform round and oval vesicular nuclei with conspicuous nucleoli and a small amount of pale cytoplasm. These are arranged in irregularly shaped solid islands which are haphazardly dispersed in a dense fibrous stroma. A palisaded arrangement of tumour cells at the periphery of these islands is noted. Occasional mitoses are identified. No necrosis is seen. Immunohistochemistry shows strong staining in tumour cells for pancytokeratin AE1/AE3, BerEp4, synaptophysin, CDX2 and neurone specific enolase (NSE) and moderate staining for CD56. No staining for S-100 protein or TTF-1 is seen in tumour cells. The features are of carcinoid tumour. The topoisomerase labelling index is approximately 3%

FINAL DIAGNOSIS:Right orbital tumour: Carcinoid tumour

Primary carcinoid tumours of the orbit are rare. Those that have been reported have usually had a dural attachment. Metastatic carcinoid tumours usually have atypical features which this tumour lacks. Primary sites that should be excluded however include lung, caecum/appendix, small bowel and pancreas.

CT

CT Abdo/Pelvis following histology

2 cm lesion is seen in the mid-distal ileum, 50 to 60 cm from the ileocaecal valve. This is associated with a very short segment intussusception. Adjacent mesenteric masses, the largest measuring 2.2 x 1.2 x 2.0 cm, shows mild desmoplastic reaction.

No para-aortic, retroperitoneal and inguinal or thoracic lymphadenopathy. An enhancing lesion in hepatic segment 7 measures 1.9 x 1.5 x 1.3 cm. No further liver lesions are seen. Multiple small soft tissue nodules are seen from the gallbladder wall, likely polyps. No abnormality of the kidneys, spleen, pancreas, adrenals or bladder. No suspicious expansile or destructive osseous lesions.

Conclusion: 

Ileal lesion, with adjacent desmoplastic mesenteric mass is concerning for a carcinoid tumour. Local mesenteric lymphadenopathy.

Enhancing hepatic lesion may represent a carcinoid metastasis, with the differential being a haemangioma. This could be further evaluated with contrast enhanced ultrasound or quad-phase CT.

Pathology

Histology (bowel)

MACROSCOPIC DESCRIPTION:

The specimen is a 300mm length of small bowel 20mm in diameter. Attached is mesentery along its entire length up to 50mm in thickness and 30mm in width. A polypoid brown tan nodule 35x25x15mm arises within the small bowel lumen. The nodule appears to push into but not through muscularis propria. The small bowel serosa is not affected and the lesion is 122mm from the closest resection margin. Multiple nodes are present in the small bowel mesentry.

MICROSCOPIC DESCRIPTION:

Sections show a neuroendocrine carcinoma extending into and focally through the muscularis propria. The tumour is composed of variably sized nests of mono-morphic polygonal cells with round nuclei and vesicular chromatin. There are up to 28 mitoses per 10 high power fields. There is no necrosis. Small nests are seen infiltrating through the muscularis propria and into adventitia with focal involvement of the serosal surface. Extensive lymphovascular invasion is identified. There is no perineural invasion. The mucosal margins are not involved by tumour. Metastatic tumour is present in 6 of 14 lymph nodes, including the apical lymph node. There are mutliple foci of extra-nodal extension. 

FINAL DIAGNOSIS: 

Well differentiated neuroendocrine carcinoma (AJCC pT4, pN2, M1 Stage IV 2).

This tumour has been compared with the previous orbital mass and has the same morphological appearance. The findings are consistent with a primary small bowel neuroendocrine carcinoma metastastic to the orbit.

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Case information

rID: 28165
Case created: 13th Mar 2014
Last edited: 30th Aug 2017
Inclusion in quiz mode: Included

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