| Session: Extramedullary manifestation of neoplastic myeloid disorder |
| Case number: 071 |
Submitter(s): Erik A. Ranheim, Catherine P. Leith.
Clinical history
The patient is a 42 y.o. M with a history of sarcoidosis and a hobby of collecting old beer cans by digging in abandoned cabins in Northern Wisconsin presented with a left neck mass. Excisional biopsy was most consistent with myeloid sarcoma. Bone marrow biopsies have been negative. Imaging revealed lung lesions, pericardial lymphadenopathy, and masslike lesions in the spleen. Core biopsy of the spleen revealed extensive caseating granulomas without apparent organisms and no evidence of neoplasm. Urinary histoplasma antigen was strongly positive. Treatment with itraconazole resulted in decreased urinary antigen, but the patient continued to feel poorly and the spleen remained enlarged. Splenectomy 6 months after presentation again showed caseating granulomas and no malignancy. Adenopathy of the neck and mediastinum persisted. Biopsy of a left cervical lymph node agaom showed findings most compatible with myelocytic sarcoma with histiocytic differentiation. The patient was treated with autologous transplant and has done well.
Details of gross/microscopic pathology:
The original neck mass biopsy (2005-1) revealed near complete effacement of nodal architecture with monotonous sheets of atypical mononuclear cells with abundant pink cytoplasm, irregular nuclear contours, and distinct nucleoli. Zonal necrosis was present. The repeat cervical node biopsy (2005-2) performed 9 months later revealed a parafollicular and sinusoidal distribution of more frankly atypical appearing mononuclear cells with eosinophilic granular cytoplasm and very prominent eosinophilic nucleoli. Splenic core biopsy and splenectomy (320 g) specimens showed extensive caseating granulomata without cytologic atypia. AFB and GMS staining revealed no apparent organisms.
Immunophenotype (flow cytometry/immunohistochemistry):
Immunohistochemical staining of the initial neck mass revealed that the atypical cells expressed CD45, CD68, CD43, and lysozyme. Myeloperoxidase was noted only in scattered cells. The tumor cells did not express CD3, CD20, CD5, CD21, CD30, CD15, CD138, S100, CD1a, CD34, CD117, or CMV antigens. The second biopsy showed similar immunoreactivity but failed to show any MPO staining and was positive for monocyte/macrophage marker CD163. Unfortunately, material was not available for flow cytometric analysis.
Cytogenetics:
N/A.
Molecular analysis:
N/A.
Interesting feature(s) of submitted case:
This case is atypical in its histologic appearance, with an altered appearance between biopsies. Despite a long period where the patient remained untreated for his hematologic neoplasm (while being treated for presumed fungal infection), the tumor remained confined to cervical nodes, never involving the bone marrow to the best of our knowledge. In addition, the confounding factor of histiocyte-dominated lesions due to infection and potentially to sarcoidosis added a layer of difficulty both pathologically and clinically.
Proposed diagnosis:
Myeloid sarcoma with histiocytic differentiation.
Panel diagnosis:
differential diagnosis includes monocytic sarcoma and histiocytic sarcoma
Comments:
Stains performed by the panel: MPO-, CD68PGM1+, NPM- (+in nuclei)
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