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01-12-2022 | Sarcoma | Review

Pseudomyogenic hemangioendothelioma with bone and soft tissue involvement with favorable response to pamidronate: a case report and systematic review of the literature

Authors: María Lorena Brance, Nicolás M. Cóccaro, Pablo Roitman, Alejandro Castiglioni, Florencia Agostinis, Mariel Spense, Bárbara Scheitlin, Nicholas Rene, Lucas R. Brun

Published in: Archives of Osteoporosis | Issue 1/2022

Abstract

Pseudomyogenic hemangioendothelioma (PMH) can be a challenge for diagnosis and might be confused with other tumors, such as epithelioid sarcoma. Here we present a case and a systematic review of the literature to identify and discuss PMH treatment in primary bone involvement. A 25-year-old woman was referred for bone pain (10/10) in the left lower limb. Magnetic resonance imaging (MRI) showed multiple bone lesions (left femur, tibia, patella, ankle, and foot) with well-defined borders without signs of local aggressiveness. Positron Emission Tomography-Computed Tomography (PET-CT) showed multiple metabolic musculoskeletal lesions in the left lower limb. A CT scan–guided biopsy was performed. Histological and immunohistochemical findings confirmed the diagnosis of PMH. After treatment with intravenous pamidronate (90 mg/monthly), the patient had clinical improvement, mild pain 2/10 without the use of non-steroidal anti-inflammatory drugs or opiates. Follow-up was assessed by MRI and PET-CT. PET-CT showed metabolic resolution of most of the bone and muscular lesions and a significant improvement of the femoral lesion. MRI showed that the lesions in the left femur, tibia, and foot had a marked decrease in size without intravenous post-contrast enhancement and smaller lesions had disappeared. After a 3-year follow-up, PET-CT showed no metabolically active images. Literature review identified 31 records including 58 clinical cases of PMH with primary bone involvement and treatment description for qualitative analysis. Most lesions (69%) were treated by local excision or curettage. In addition, amputations were performed in a significant percentage of cases (20.7%). In the last years, mTOR inhibitors (n = 7) and anti-resorptive treatments (n = 4) were considered as alternative treatment options, especially in multifocal lesions.

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Title: Pseudomyogenic hemangioendothelioma with bone and soft tissue involvement with favorable response to pamidronate: a case report and systematic review of the literature
Authors: María Lorena Brance
Nicolás M. Cóccaro
Pablo Roitman
Alejandro Castiglioni
Florencia Agostinis
Mariel Spense
Bárbara Scheitlin
Nicholas Rene
Lucas R. Brun
Publication date: 01-12-2022
Publisher: Springer London
Keywords: Sarcoma
mTOR-Inhibitors
Magnetic Resonance Imaging
Magnetic Resonance Imaging
Published in: Archives of Osteoporosis / Issue 1/2022
Print ISSN: 1862-3522
Electronic ISSN: 1862-3514
DOI: https://doi.org/10.1007/s11657-022-01062-4

At a glance: The STEP trials

Springer Medicine

Pseudomyogenic hemangioendothelioma with bone and soft tissue involvement with favorable response to pamidronate: a case report and systematic review of the literature

Abstract

At a glance: The STEP trials

Springer Medicine

Abstract

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