ALK-positive anaplastic large cell lymphoma with primary bone involvement in children.

Am J Clin Pathol. 2006 Jan;125(1):57-63. Related Articles, Links 

ALK-positive anaplastic large cell lymphoma with primary bone
involvement in children.

NA, Ross CW, Finn WG, Valdez R, Ruiz R, Koujok K, Schnitzer B.

Department of Pathology, Oklahoma University Health Sciences Center,
Oklahoma City, USA.

We describe the clinical, radiologic, and pathologic features of
primary bone anaplastic large cell lymphoma (ALCL) in 3 boys.
Radiologic imaging showed lytic lesions involving sacrum, femur, or
rib. Bone was the only site of disease in 2 cases; an associated
partial lymph node was involved in case 3. Differential diagnoses
included osteomyelitis and small round cell tumors of childhood,
particularly Ewing sarcoma. Preoperatively, ALCL was not a diagnostic
consideration in any case. Two cases showed classic large pleomorphic
cells; 1 showed a composite pattern with a distinct small cell
component and the more typical large cell type. Neoplastic cells in all
cases showed strong CD30 and anaplastic lymphoma kinase expression with
relatively weak epithelial membrane antigen positivity. Cytotoxic
granule protein was expressed in 2 cases. All cases showed unusually
strong expression of neuron-specific enolase (NSE). Two patients were
disease-free at last follow-up (15 months and 11 years); 1 patient died
of disseminated disease within a year of diagnosis. ALCL should be
considered a diagnostic possibility when evaluating neoplastic bone
lesions in children. Although expression of NSE in ALCL has not been
emphasized in the literature, it is worth noting because it may pose a
diagnostic pitfall.

PMID: 16482992 [PubMed – in process]

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