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Case Reports
. 2018 Jan 30:8:1.
doi: 10.1186/s13569-018-0087-9. eCollection 2018.

Intraosseous papillary intralymphatic angioendothelioma (PILA): one new case and review of the literature

Marco Gambarotti  1 , Alberto Righi  1   2 , Marta Sbaraglia  3 , Giuseppe Bianchi  4 , Piero Picci  1 , Daniel Vanel  1 , Angelo Paolo Dei Tos  1   3   5
Affiliations
Case Reports

Intraosseous papillary intralymphatic angioendothelioma (PILA): one new case and review of the literature

Marco Gambarotti et al. Clin Sarcoma Res. .

Abstract

Background: Papillary intralymphatic angioendothelioma (PILA) is a locally aggressive, rarely metastasizing vascular tumor, generally occurring in the soft tissues, with less than 40 cases described in the literature and only three cases reported in bone.

Case presentation: We describe the case of a 51-year-old male with an intraosseous PILA of the proximal edge of his left clavicle and two other lesions evident on imaging. The patient was treated with marginal resection of the clavicle lesion but was lost to follow-up 1 month after surgery.

Conclusions: PILA can also occur in bone, albeit very rarely, and has to be considered in the differential diagnosis of vascular bone tumors.

Keywords: Bone; Dabska tumor; Papillary intralymphatic angioendothelioma.

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Figures

Fig. 1
Fig. 1
On CT A the lesion (see arrows) involves the medial end of the clavicle and is purely lytic; the cortex is destroyed and soft tissues are invaded. On axial MRI the signal is isointense on T1-weighted (B), and heterogeneously hyperintense on fat-saturated T2-weighted (C) and after contrast medium injection (D)
Fig. 2
Fig. 2
Gross features A an 8 cm. Lesion of the left clavicle, with soft tissue invasion. Histological features of the lesion, composed of glomerulus-like structures (B), with papillary structures projecting into the lumen, covered by plump “hobnail” endothelial cells (C–E). Immunohistochemical positivity for podoplanin (D2-40) in the neoplastic cells (F)
See this image and copyright information in PMC

References

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