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Case Reports
. 2017 Oct 25;5(12):1988-1991.
doi: 10.1002/ccr3.1232. eCollection 2017 Dec.

Multiple large osteolytic lesions in a patient with systemic mastocytosis: a challenging diagnosis

Massimiliano Bonifacio  1   2 , Roberta Zanotti  1   2 , Emanuele Guardalben  1 , Elda Mimiola  1 , Francesca Scognamiglio  3 , Omar Perbellini  3 , Giovanna De Matteis  4 , Luis Escribano  5   6 , Patrizia Bonadonna  2   7 , Daniela Grigolato  8 , Sergio Bissoli  9 , Alice Parisi  10 , Alberto Zamò  10 , Achille Ambrosetti  1 , Maurizio Rossini  2   11
Affiliations
Case Reports

Multiple large osteolytic lesions in a patient with systemic mastocytosis: a challenging diagnosis

Massimiliano Bonifacio et al. Clin Case Rep. .

Abstract

Patients with advanced variants of Systemic Mastocytosis may develop destructive bone lesions when massive mast cell (MC) infiltrates are present. Finding of large osteolyses in indolent systemic mastocytosis, typically characterized by low MC burden, should prompt investigations for an alternative explanation.

Keywords: Non‐hodgkin lymphoma; osteolysis; primary bone lymphoma; systemic mastocytosis; tryptase.

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Figures

Figure 1
Figure 1
Histopathology and immunohistochemistry of the first bone marrow biopsy (upper panel, A–D) and of the lytic lesion (lower panel, E–H). In the first biopsy, H&E staining shows hypercellular areas (A) that correspond to CD117‐positive mast cell aggregates (B), which show co‐expression of CD25 (C). CD138 (D) shows an increase in the plasma cell population. In the TC‐guided rebiopsy, H&E shows a dense sheet of large cells (E) expressing the B‐cell marker CD20 (F) as well as CD10 (G). The proliferation index as demonstrated by Ki‐67 staining (H) is very high.
Figure 2
Figure 2
Axial and coronal fused FDG‐PET/CT images in the same patient before treatment (A) showed hypermetabolic bone lesions in both humeri, in some ribs, in a lumbar vertebra, in the pelvis (SUV max 34), and in the left femur. After treatment (B), the FDG uptake in the same areas was normal. Corresponding CT images depicted lytic bone lesions.
See this image and copyright information in PMC

References

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