Pseudomyogenic Hemangioendothelioma: A Vascular Tumor Previously Undescribed in the Oral Cavity

Rawal, Yeshwant B.; Anderson, Kenneth M.; Dodson, Thomas B.

doi:10.1007/s12105-016-0770-1

Cited by 20 publications

(35 citation statements)

References 17 publications

(39 reference statements)

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“…On the other hand, there is not expression of CD34, S‐100, smooth muscle actin, desmin, MyoD1, and HMB45 (Fan et al, ; Rawal, Anderson, & Dodson, ; Requena et al, ).…”

Section: Discussionmentioning

confidence: 99%

“…Nuclear atypia can be mild to moderate. In some cases a neutrophilic inflammatory infiltrate can be observed.Immunohistochemically, tumor cells show diffuse strong expression of Factor VIII, FLI-1, INI-1, vimentin, MDM2, CDK4, local expression of CD31, cytokeratin AE1/AE3, EMA, and P63.On the other hand, there is not expression of CD34, S-100, smooth muscle actin, desmin, MyoD1, and HMB45(Fan et al, 2015;Rawal, Anderson, & Dodson, 2017;Requena et al, 2013).Vimentin has a low-grade specificity and for this reason is no longer relevant(Rawal et al, 2017).Diagnosis of PMH is based on the peculiar clinical presentation, histological examination, and demonstration of the expression of both keratins and endothelial markers by immunohistochemistry.Molecular genetics of PMH is unknown: Trombetta et al described a balanced translocation between Chromosomes 7 and 19 [t(7;19) (q22;q13)] as a unique clonal change(Trombetta et al, 2011). In a recent study, Hung et al describes a new useful diagnostic marker for PMH.…”

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confidence: 99%

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The management of pseudomyogenic hemangioendothelioma of the foot: A case report and review of the literature

Pranteda

Magri

Mellai

et al. 2018

Dermatologic Therapy

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Pseudomyogenic hemangioendothelioma (PMH) is a rare, mostly indolent, endothelial neoplasm of low‐grade malignancy, often mimicking myoid and epithelioid tumors histologically. It is more frequent in young adult males and it usually presents with multiple cutaneous nodules, mostly localized at the extremities. It traverses several tissue planes simultaneously and can involve dermis, subcutis, skeletal muscle, and bone. Histologically, it is characterized by plump spindle cells with eosinophilic cytoplasm, often arranged in fascicles and epithelioid cells with “pseudomyogenic” morphology. Immunohistochemically, PMH is positive for Factor VIII, FLI‐1, INI‐1, vimentin, MDM2, CDK4, CD31, AE1/AE3, EMA, and P63. The efficacy of treatments is only partially known. Because of the frequent multifocal aspect of PMH, which contraindicates surgery, systemic treatments, such as gemcitabine, sirolimus, and everolimus are used. Based on our observation of multifocal PMH of the foot in a 17‐year‐old male patient, treated with gemcitabine with complete cutaneous response in a 2‐year follow‐up, we decided to discuss this rare tumor and underline its progression and therapeutic approaches. Thanks to a correct diagnosis, it is possible to avoid aggressive therapeutic approaches, which would be necessary for nonindolent diseases, such as sarcoma, which often needs amputation.

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“…On the other hand, there is not expression of CD34, S‐100, smooth muscle actin, desmin, MyoD1, and HMB45 (Fan et al, ; Rawal, Anderson, & Dodson, ; Requena et al, ).…”

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

The management of pseudomyogenic hemangioendothelioma of the foot: A case report and review of the literature

Pranteda

Magri

Mellai

et al. 2018

Dermatologic Therapy

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“…This rare tumor type typically occurs in the lower extremities of young to middle-aged adults with a striking male predominance [2]. Less commonly, it may involve upper extremities, trunk, pelvis, and head and neck region [3, 4]. Multicentric synchronous presentation with involvement of different tissue planes in an anatomic region is a common feature of PMHE.…”

Section: Introductionmentioning

confidence: 99%

Primary pseudomyogenic hemangioendothelioma of the vulva: a rare location for a rare entity

Sun

Wang

2019

Diagn Pathol

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Background: Pseudomyogenic hemangioendothelioma (PMHE) is a recently described vascular neoplasm which typically occurs in the lower extremities of young to middle-aged adults. Case presentation: We present here a unique case of PMHE arising primarily in the vulva of a 51-year-old woman who presented with a painful vulvar nodule. Clinically, it was thought as Bartholin gland cyst, vulvar hematoma or papilloma. On surgery, two nodules were found with one located in the superficial dermis and the other in the deep subcutis. Histologically, these two nodules showed similar features, composed of fascicles or sheets of plump spindled to epithelioid cells with eosinophilic cytoplasm. Given the morphological resemblance to a myogenic tumor, the lesion was initially diagnosed as a rhabdomyosarcoma by the referring pathologist. However, a comprehensive reevaluation of the submitted slides made us reconsider a PMHE, which was subsequently confirmed by immunohistochemical study. Conclusion: This case demonstrates that PMHE can also develop in the female external genitalia albeit extremely rare. This disease should be included in the differential diagnostic list of vulvar tumors with spindled to epithelioid morphology and cytokeratin-positive immunophenotypes.

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“…Pseudomyogenic Hemangioendothelioma (Epithelioid Sarcoma-like Haemangioendothelioma) is a rare soft tissue tumor of vascular origin. [1] It is a distinctive, locally aggressive, rarely metastasizing tumor having indolent behaviour. [1,2,3] The neoplasm occurs more frequently in young adult males.…”

Section: Introductionmentioning

confidence: 99%

“…[1] It is a distinctive, locally aggressive, rarely metastasizing tumor having indolent behaviour. [1,2,3] The neoplasm occurs more frequently in young adult males. [2,4] It usually affects the lower limbs.…”

Section: Introductionmentioning

confidence: 99%

Pseudomyogenic Hemangioendothelioma (Epithelioid Sarcoma-like Haemangioendothelioma) - Case Report of a Rare Entity

Kumarguru¹,

N²,

R³

et al. 2019

APALM

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Pseudomyogenic Hemangioendothelioma (Epithelioid Sarcoma-like Haemangioendothelioma) is a rare soft tissue tumor of endothelial origin. It is a distinctive neoplasm having indolent behaviour. A 27-year-old male presented with history of multiple small ulcers over right shoulder region since 2 months. On examination, diffuse indurated plaques were seen over the right shoulder with multiple ulcers. A Provisional diagnosis of non-healing ulcer over right shoulder was offered. Pus culture was positive for coagulase negative Staphyloccus species. The lesion was excised and sent for histopathology. Microscopy showed ulcerated epidermis. Subepithelium showed tumor tissue composed of cells arranged in fascicles, sheets and irregular nodules. Individual tumor cells were oval to spindle shaped cells having brightly eosinophilic cytoplasm and vesicular nucleus with noticeable nucleoli. At places, endothelial cell proliferation and neovascularization was seen. Possibility of epithelioid sarcoma, epithelioid hemangioendothelioma and malignant melanoma was considered. Immunohistochemistry was performed. Neoplastic cells were positive for vimentin, CD31, CK19 and negative for CD34, HMB45 and EMA. A final diagnosis of Epithelioid sarcoma-like Hemangioendothelioma (Pseudomyogenic Hemangioendothelioma) was offered. It is an unusual pathological entity with an unique clinical presentation. Histopathology is challenging. Immunohistochemistry is not only confirmatory, but also mandatory to solve the diagnostic dilemma.

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Pseudomyogenic Hemangioendothelioma: A Vascular Tumor Previously Undescribed in the Oral Cavity

Cited by 20 publications

References 17 publications

The management of pseudomyogenic hemangioendothelioma of the foot: A case report and review of the literature

The management of pseudomyogenic hemangioendothelioma of the foot: A case report and review of the literature

Primary pseudomyogenic hemangioendothelioma of the vulva: a rare location for a rare entity

Pseudomyogenic Hemangioendothelioma (Epithelioid Sarcoma-like Haemangioendothelioma) - Case Report of a Rare Entity

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