SH/EAHP 2007 Workshop - Progress in T-cell and NK cell Malignancies - title graphic

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Session: Extramedullary manifestation of neoplastic myeloid disorder
Case number: 173

Submitter(s): Masaru Hosone, Yuichi Sugisaki, Shotaro Maeda, Zenya Naito.

Clinical history
A 36-year-old Japanese male presentd with lower abdominal pain due to intestinal obstruction.The barium-contrast radiography of small intestine showed 3.5-cm-long ileac stenosis and laparoscopic partial resection of the ileum was performed. No superficial lymph node swelling and no hepatosplenomegaly were detected.The CBC on admission was normal;WBC:9400/ul (no blasts observed), RBC:423x104/ul,Hb:12.5g/dl,Ht:36.6%,PLT:28.6x104/ul.Chest X-ray and abdominal X-ray were normal.Bone marrow aspirates showed normocellular marrow with no leukemic blasts.The whole body CT scan revealed no visceral lymph node swelling and no other tumor masses.

Details of gross/microscopic pathology:
The resected 13-cm-long ileum was moderately expanded at the oral side due to an obstructive tumor mass situated in the distal portion.The lesion was a slightly elevated and the surface of the mucosa was partially eroded.The fresh lesion is not greenish but yellowish.Microscopically medium to large atypical lymphoid cells showed transmural infiltrate.The surface-lining epithelium was almost intact.The tumor cells showed no lympho-epithelial lesion.Tingible body macrophages were occasioanlly seen.At high power,irregular shape of nuclei and fine chromatin pattern were observed.(Fig.1-Fig.3) Under the conventional 20%-formalin-fixed H&E sections,the primary differential diagnoses were small non-cleaved cell/non-Burkitt's lymphoma and diffuse large B-cell lymphoma.

Immunophenotype (flow cytometry/immunohistochemistry):
No flow cytometric analysis of the ileac tumor for immunophenotyping was done.Immunohistochemistry of the formalin-fixed specimen of the lesion showed CD45(+),CD3(-),CD45RO(-),CD20(-),CD79a(-),CD43/MT-1(+)(Fig.4),CD68/KP-1(+)(Fig.5),MPO(+)(Fig.6).

Cytogenetics:
Not done. The imprint cytology of the ileac tumor was not prepared.

Molecular analysis:
Not done.

Interesting feature(s) of submitted case:
Myeloid/granulocytic sarcoma of small intestine/ileum is a rare manifestation of extramedullary acute myeloid leukemia in the previous reports.

Proposed diagnosis:
Myeloid sarcoma/granulocytic sarcoma of ileum.

Panel diagnosis:
agree with proposed diagnosis

Comments:
Stains performed by the panel: CD68 PGM1 -/+, CD117 +/-, NPM- (+in nuclei)

Images:
Case Image 115a.jpg Figure 1
Case Image 115b.jpg Figure 2
Case Image 115c.jpg Figure 3
Case Image 115d.jpg Figure 4
Case Image 115e.jpg Figure 5
Case Image 115f.jpg Figure 6

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