Skeletal Radiol DOI 10.1007/s00256-014-2024-1
CASE REPORT
Primary pseudomyogenic haemangioendothelioma of bone: report of two cases Alberto Righi & Marco Gambarotti & Piero Picci & Angelo Paolo Dei Tos & Daniel Vanel
Received: 13 August 2014 / Revised: 24 September 2014 / Accepted: 28 September 2014 # ISS 2014
Abstract Pseudomyogenic haemangioendothelioma (epithelioid sarcoma-like haemangioendothelioma) is a recently described, rarely metastasising neoplasm of the soft tissues that often presents as multiple discontinuous nodules in different tissue planes of a limb. This tumour type is extremely difficult to diagnose because no morphological evidence suggestive of endothelial differentiation is present to confirm a radiological suspicion of vascular neoplasm. All but one case published in the literature underline that bone involvement is secondary to deep and superficial soft tissue nodules. Here, we report two cases of primary bone pseudomyogenic haemangioendothelioma occurring in the radius and cuboid bone with a multifocal presentation.
current WHO classification of soft tissue tumours [4], PMH is listed as an intermediate, rarely metastasising, vascular tumour with peculiar clinical and pathological features [1]. PMH has a propensity to occur in the lower limbs of young male patients and to be multicentric with involvement of different tissue planes. Since the first description, fewer than 65 cases have been published in the literature [2–8], but only one primary was in bone [7]. All but one case, which reported bone involvement, were PMHs occuring in deep and superficial soft tissue with secondary bone involvement [2–8]. Here we report two primary bone PMHs occurring in the radius of a 25-year-old male and in the cuboid bone of a 66year-old female, both with a multifocal presentation.
Keywords Pseudomyogenic haemangioendothelioma . Bone tumour . Radius . Multicentric . Vascular neoplasm
Case reports Case 1
Introduction Pseudomyogenic haemangioendothelioma (PMH), also sometimes known as epithelioid sarcoma-like haemangioendothelioma [1], is a soft tissue tumour entity, recently described for the first time by Hornick and Fletcher in 2011 [2] as having the same morphological features as the “fibroma-like, fibrohistiocytic/myxoid variant” of epithelioid sarcoma described in five patients by Mirra et al. [3]. In the A. Righi (*) : M. Gambarotti : P. Picci : A. P. Dei Tos : D. Vanel Pathology Department, Rizzoli Institute, Via del Barbiano 1/10, 40136 Bologna, Italy e-mail: [email protected] A. P. Dei Tos Department of Pathology, Treviso Regional Hospital, Treviso, Italy
During the histological review of primitive spindle/ pleomorphic sarcoma of bone in our database on the basis of the more recent diagnostic techniques that changed the classification of soft tissue sarcomas, we reclassified a case of fibrosarcoma as primary PMH of the bone [9]. A previously healthy 25-year-old male, Caucasian, was referred to our institution in 1956 for pain in the left wrist that had been present for 3 months without a trauma history. Radiographs of the forearm showed a 6-cm osteolytic lesion that involved the medial cortex of the distal third of the left radius without periosteal reaction. Proximally and distally to this lesion, two other well-delimitated osteolytic areas were evident (Fig. 1). An open biopsy was performed on the two largest lesions, and the diagnosis of PMH was confirmed on review of the original material obtained from both biopsy sites.
Skeletal Radiol
neoplastic cells were strongly positive to the vascular marker ERG (Fig. 2c) and smooth muscle actin and focally positive to pan-cytokeratin AE1/AE3 (Fig. 2d). Protein S-100, CD34 and h-caldesmon were negative. Resection of the medium-inferior third of the radius was done with a calf rib transplantation. Six months after the surgical treatment (October 1956), multiple nodular subcutaneous lesions appeared in the posterior region of the left forearm and were removed with the diagnosis of multiple recurrences. Fourteen months after the first surgical treatment, the patient underwent an amputation of the inferior third of the left arm because of multiple nodules infiltrating the forearm muscles associated with a severe functional deficit and muscle hypotrophy. At the last follow-up, the patient was alive without disease 19 years after the amputation. Case 2 Fig. 1 AP radiograph of the forearm reveals three well-delineated osteolytic lesions of the left radius without periosteal reaction
According to haematoxylin and eosin staining, the tumour was composed of sheets and loose fascicles of plump spindle cells with abundant brightly eosinophilic cytoplasm, sometimes mimicking rhabdomyoblasts, which infiltrated the bony trabeculae. The tumour cells contained vesicular nuclei with small nucleoli and were associated with scattered neutrophils (Fig. 2a–b). Mitoses were rare (
CASE REPORT
Primary pseudomyogenic haemangioendothelioma of bone: report of two cases Alberto Righi & Marco Gambarotti & Piero Picci & Angelo Paolo Dei Tos & Daniel Vanel
Received: 13 August 2014 / Revised: 24 September 2014 / Accepted: 28 September 2014 # ISS 2014
Abstract Pseudomyogenic haemangioendothelioma (epithelioid sarcoma-like haemangioendothelioma) is a recently described, rarely metastasising neoplasm of the soft tissues that often presents as multiple discontinuous nodules in different tissue planes of a limb. This tumour type is extremely difficult to diagnose because no morphological evidence suggestive of endothelial differentiation is present to confirm a radiological suspicion of vascular neoplasm. All but one case published in the literature underline that bone involvement is secondary to deep and superficial soft tissue nodules. Here, we report two cases of primary bone pseudomyogenic haemangioendothelioma occurring in the radius and cuboid bone with a multifocal presentation.
current WHO classification of soft tissue tumours [4], PMH is listed as an intermediate, rarely metastasising, vascular tumour with peculiar clinical and pathological features [1]. PMH has a propensity to occur in the lower limbs of young male patients and to be multicentric with involvement of different tissue planes. Since the first description, fewer than 65 cases have been published in the literature [2–8], but only one primary was in bone [7]. All but one case, which reported bone involvement, were PMHs occuring in deep and superficial soft tissue with secondary bone involvement [2–8]. Here we report two primary bone PMHs occurring in the radius of a 25-year-old male and in the cuboid bone of a 66year-old female, both with a multifocal presentation.
Keywords Pseudomyogenic haemangioendothelioma . Bone tumour . Radius . Multicentric . Vascular neoplasm
Case reports Case 1
Introduction Pseudomyogenic haemangioendothelioma (PMH), also sometimes known as epithelioid sarcoma-like haemangioendothelioma [1], is a soft tissue tumour entity, recently described for the first time by Hornick and Fletcher in 2011 [2] as having the same morphological features as the “fibroma-like, fibrohistiocytic/myxoid variant” of epithelioid sarcoma described in five patients by Mirra et al. [3]. In the A. Righi (*) : M. Gambarotti : P. Picci : A. P. Dei Tos : D. Vanel Pathology Department, Rizzoli Institute, Via del Barbiano 1/10, 40136 Bologna, Italy e-mail: [email protected] A. P. Dei Tos Department of Pathology, Treviso Regional Hospital, Treviso, Italy
During the histological review of primitive spindle/ pleomorphic sarcoma of bone in our database on the basis of the more recent diagnostic techniques that changed the classification of soft tissue sarcomas, we reclassified a case of fibrosarcoma as primary PMH of the bone [9]. A previously healthy 25-year-old male, Caucasian, was referred to our institution in 1956 for pain in the left wrist that had been present for 3 months without a trauma history. Radiographs of the forearm showed a 6-cm osteolytic lesion that involved the medial cortex of the distal third of the left radius without periosteal reaction. Proximally and distally to this lesion, two other well-delimitated osteolytic areas were evident (Fig. 1). An open biopsy was performed on the two largest lesions, and the diagnosis of PMH was confirmed on review of the original material obtained from both biopsy sites.
Skeletal Radiol
neoplastic cells were strongly positive to the vascular marker ERG (Fig. 2c) and smooth muscle actin and focally positive to pan-cytokeratin AE1/AE3 (Fig. 2d). Protein S-100, CD34 and h-caldesmon were negative. Resection of the medium-inferior third of the radius was done with a calf rib transplantation. Six months after the surgical treatment (October 1956), multiple nodular subcutaneous lesions appeared in the posterior region of the left forearm and were removed with the diagnosis of multiple recurrences. Fourteen months after the first surgical treatment, the patient underwent an amputation of the inferior third of the left arm because of multiple nodules infiltrating the forearm muscles associated with a severe functional deficit and muscle hypotrophy. At the last follow-up, the patient was alive without disease 19 years after the amputation. Case 2 Fig. 1 AP radiograph of the forearm reveals three well-delineated osteolytic lesions of the left radius without periosteal reaction
According to haematoxylin and eosin staining, the tumour was composed of sheets and loose fascicles of plump spindle cells with abundant brightly eosinophilic cytoplasm, sometimes mimicking rhabdomyoblasts, which infiltrated the bony trabeculae. The tumour cells contained vesicular nuclei with small nucleoli and were associated with scattered neutrophils (Fig. 2a–b). Mitoses were rare (
