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Primary Pseudomyogenic Hemangioendothelioma of Bone.

Inyang, Alero; Mertens, Fredrik LU ; Puls, Florian; Sumathi, Vaiyapuri; Inwards, Carrie; Folpe, Andrew; Lee, Cheng-Han; Zhang, Yaxia; Symmans, Pennie and Rubin, Brian, et al. (2016) In American Journal of Surgical Pathology 40(5). p.587-598
Abstract
Pseudomyogenic hemangioendothelioma (PMH) is a well-recognized neoplasm that usually arises in the soft tissue; concurrent bone involvement occurs in 24% of cases. PMH of bone without soft tissue involvement is rare. We describe the clinicopathologic findings of 10 such cases, the largest series reported to date. The study included 9 male and 1 female patient; their ages ranged from 12 to 74 years (mean 36.7 y). All patients had multiple tumors with a distinct regional distribution: 45% restricted to the lower extremity; 25% to the spine and pelvis; and 15% to the upper extremity. On imaging studies the tumors were well circumscribed and lytic. The neoplasms were composed of spindled cells arranged in intersecting fascicles with scattered... (More)
Pseudomyogenic hemangioendothelioma (PMH) is a well-recognized neoplasm that usually arises in the soft tissue; concurrent bone involvement occurs in 24% of cases. PMH of bone without soft tissue involvement is rare. We describe the clinicopathologic findings of 10 such cases, the largest series reported to date. The study included 9 male and 1 female patient; their ages ranged from 12 to 74 years (mean 36.7 y). All patients had multiple tumors with a distinct regional distribution: 45% restricted to the lower extremity; 25% to the spine and pelvis; and 15% to the upper extremity. On imaging studies the tumors were well circumscribed and lytic. The neoplasms were composed of spindled cells arranged in intersecting fascicles with scattered epithelioid cells; epithelioid cells predominated in 3 cases. The neoplastic cells contained abundant densely eosinophilic cytoplasm and vesicular nuclei. There was limited cytologic atypia and necrosis, few mitoses (0 to 2/10 high-power fields), and inconspicuous stroma. Unique findings included abundant intratumoral reactive woven bone and hemorrhage with numerous osteoclast-like giant cells. Immunohistochemically, most tumors were positive for keratin, ERG, and CD31; CD34 was negative. The balanced t(7:19)(q22;13) translocation was documented in 3 cases. Follow-up is limited, but no patient developed documented visceral dissemination, and all have stable or progressive osseous disease. PMH exclusively involving bone is rare. It is multicentric, often involves the lower extremity, and has unusual morphology. The differential diagnosis includes epithelioid vascular neoplasms, giant cell tumor, bone forming neoplasms, and metastatic carcinoma. Because of its rarity, unusual presentation, and morphology, accurate diagnosis can be challenging. (Less)
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American Journal of Surgical Pathology
volume
40
issue
5
pages
587 - 598
publisher
Lippincott Williams & Wilkins
external identifiers
  • pmid:26872012
  • scopus:84957900667
ISSN
1532-0979
DOI
10.1097/PAS.0000000000000613
language
English
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yes
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f313754d-70ac-4019-8a97-801630776bf2 (old id 8825653)
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http://www.ncbi.nlm.nih.gov/pubmed/26872012?dopt=Abstract
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2016-03-04 10:43:41
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@article{f313754d-70ac-4019-8a97-801630776bf2,
  abstract     = {Pseudomyogenic hemangioendothelioma (PMH) is a well-recognized neoplasm that usually arises in the soft tissue; concurrent bone involvement occurs in 24% of cases. PMH of bone without soft tissue involvement is rare. We describe the clinicopathologic findings of 10 such cases, the largest series reported to date. The study included 9 male and 1 female patient; their ages ranged from 12 to 74 years (mean 36.7 y). All patients had multiple tumors with a distinct regional distribution: 45% restricted to the lower extremity; 25% to the spine and pelvis; and 15% to the upper extremity. On imaging studies the tumors were well circumscribed and lytic. The neoplasms were composed of spindled cells arranged in intersecting fascicles with scattered epithelioid cells; epithelioid cells predominated in 3 cases. The neoplastic cells contained abundant densely eosinophilic cytoplasm and vesicular nuclei. There was limited cytologic atypia and necrosis, few mitoses (0 to 2/10 high-power fields), and inconspicuous stroma. Unique findings included abundant intratumoral reactive woven bone and hemorrhage with numerous osteoclast-like giant cells. Immunohistochemically, most tumors were positive for keratin, ERG, and CD31; CD34 was negative. The balanced t(7:19)(q22;13) translocation was documented in 3 cases. Follow-up is limited, but no patient developed documented visceral dissemination, and all have stable or progressive osseous disease. PMH exclusively involving bone is rare. It is multicentric, often involves the lower extremity, and has unusual morphology. The differential diagnosis includes epithelioid vascular neoplasms, giant cell tumor, bone forming neoplasms, and metastatic carcinoma. Because of its rarity, unusual presentation, and morphology, accurate diagnosis can be challenging.},
  author       = {Inyang, Alero and Mertens, Fredrik and Puls, Florian and Sumathi, Vaiyapuri and Inwards, Carrie and Folpe, Andrew and Lee, Cheng-Han and Zhang, Yaxia and Symmans, Pennie and Rubin, Brian and Nielsen, Gunnlaugur P and Nguyen, Van-Hung and Rosenberg, Andrew E},
  issn         = {1532-0979},
  language     = {eng},
  month        = {02},
  number       = {5},
  pages        = {587--598},
  publisher    = {Lippincott Williams & Wilkins},
  series       = {American Journal of Surgical Pathology},
  title        = {Primary Pseudomyogenic Hemangioendothelioma of Bone.},
  url          = {http://dx.doi.org/10.1097/PAS.0000000000000613},
  volume       = {40},
  year         = {2016},
}