| Session: Extramedullary manifestation of neoplastic myeloid disorder |
| Case number: 151 |
Submitter(s): Stefano A. Pileri, Elena Sabattini, Cristina Campidelli, Claudio Agostinelli, Francesco Bacci.
Clinical history
A 67 year old man suffering from diabetes, was operated in January 2005 for a right inguinal hernia, which relapsed the following September needing a second surgical procedure. Soon after, a rapidly growing mass was observed at the site of surgical wound initially interpreted as exuberant scar. Due to the progressive growth of this mass and the development of fever, he was hospitalized in January 2006 and a biopsy was performed (submitted for the workshop). Peripheral blood: Hb 7.2g/dL, WBC 32.000/mm3 with neutrophylia, platelets 335.000/mm3.
After diagnosis, a marrow aspirate and biopsy were performed, which turned out negative for neoplastic involvement, but showed mild dyserythropoiesis with macrocytic features on the aspirate.
On cytogenetic grounds, a 5-monosomy was detected in the marrow sample.
A total body CAT scan confirmed the presence of a 20 cm-across mass in the context of the abdominal wall, provided with extensive necrosis, which reached the small pelvis, compressed bladder and colon, with femoral thrombosis.
Chemotherapy was initiated in January (3+7 aracytin and daunoblastin): in spite of initial regression, the inguinal mass rapidly grew ulcerating the skin with massive bleeding.
The patient underwent surgery for debulking and haemostasis, but deceased in Febrary 2006.
Details of gross/microscopic pathology:
The biopsy was characterized by extensive necrosis and a diffuse cohesive neoplastic growth consisting of large, very pleomorphic cells, more often monucleated and provided with wide, eosinophilic cytoplasm and evident nucleoli. Some multinucleated giant cells and mitoses could also be observed. There were many granulocytes admixed.
The biopsy had been fixed in 10% buffered formalin according to Lillie for 24 hours.
Immunophenotype (flow cytometry/immunohistochemistry):
The immunohistochemical stains against the following molecules were perfomed: CD1a, CD2, CD3, CD7, CD20, CD21, CD34, CD45, CD56, CD61, CD68, CD68R, CD117, myeloperoxidase, LAT, factor VIII related antigen, S100, lysozyme, HECA, wide spectrum cytokeratins, and HMB45. The neoplastic cells were only positive for CD61 and LAT, being negative for all the remaining markers.
Cytogenetics:
Monosomy of chromosome 5 was detected in the marrow sample.
Molecular analysis:
No molecular analysis was performed.
Interesting feature(s) of submitted case:
Extramedullary myeloid tumours are rare diseases and particularily those with such cytological pleomorphism that could indeed raise the differential diagnoses with either non haematological disorders (e.g. malignant melanoma or undifferentiated carcinoma) or histiocytic/dendritic cell tumours. As a matter of fact, the majority of these highly atypical extramedullary myeloid tumours do actually show megakacaryoblastic differentiation.
Proposed diagnosis:
Myeloid sarcoma with megakacaryoblastic differentiation.
Panel diagnosis:
agree with proposed diagnosis
Comments:
Additional information from the submitter: monosomy 5 found also on the extramedullary tumor sample.
Images:
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Figure 1 |
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Figure 2 |
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