Cutaneous Epithelioid Hemangioendothelioma on the Sole of a Child

Carranza-Romero, Carmen; Molina-Ruiz, Ana María; Monroy, Carlos; Santos, Jesús Cuevas; Requena, Luis

doi:10.1111/pde.12539

Cited by 9 publications

(3 citation statements)

References 34 publications

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“…Meanwhile, imaging can be valuable for diagnosis [4]. Tumors can appear as low-density images and show mild delayed enhancement in CT. EHE is more frequently observed in soft tissues than visceral organs, and an isolated lesion is quite common [12, 13]. MRI is more diagnostic for soft tissue tumors and more useful for evaluating tumor resectability.…”

Section: Discussionmentioning

confidence: 99%

Clinical characteristics of epithelioid hemangioendothelioma: a single-center retrospective study

Zheng

et al. 2019

Eur J Med Res

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Background Epithelioid hemangioendothelioma (EHE) is a rare malignant vascular neoplasm with increasing incidence. However, its clinical characteristics remain unclear due to its low incidence. This study aimed to investigate the features of EHE. Methods Patients with EHE treated at our institution between January 2000 and June 2018 were enrolled. Data including demographic characteristics, treatment patterns, pathological results, and prognosis were collected, and a retrospective database was constructed for analysis. Results The cohort comprised 33 patients with a mean age of 48.0 ± 16.0 years. Eighteen (54.5%) patients were asymptomatic. The laboratory tests were unremarkable except in five and two patients who had increased CA 125 and CA 19-9, respectively. Twenty-one patients underwent surgery, while 12 patients underwent only biopsy. The postoperative morbidity rate was 28.6% (6/21). The anatomical sites of the primary lesions varied. Immunohistochemical staining was positive for CD34 and CD31 in most patients. Twenty-six patients (78.8%) were followed up at a range of 1–201 months, and 6 patients died during this period. The 1-, 3-, and 5-year cumulative survival rates were 96.2%, 87.0%, and 75.3%, respectively. The patients who had metastases or only underwent biopsy showed significantly higher mortality. Conclusions EHE is a rare malignant vascular tumor that can occur in any site of the body. Surgery is the primary choice of treatment, and pathologic evaluation is the gold standard for diagnosis. Metastases and unresectability are associated with poor prognosis.

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Section: Discussionmentioning

confidence: 99%

Clinical characteristics of epithelioid hemangioendothelioma: a single-center retrospective study

Zheng

et al. 2019

Eur J Med Res

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“…Subsequently, EHE has been considered to be a low-grade malignant vascular neoplasm with low metastatic potential but a high tendency to recur, although in a recent series of 30 cases of cutaneous EHE, the follow-up of 24 cases showed local recurrence in three cases and systemic metastasis in five cases, with four patients dying of the tumor, suggesting that EHE of the skin and soft tissues should be regarded as a fully malignant tumor rather than a borderline vascular neoplasm, although with a better prognosis than conventional angiosarcoma. In the recently revised World Health Organization classification of soft-tissue tumors, it has been placed under the heading of intermediate endothelial tumors [ 5 ]. Based on the above considerations, it could be difficult to identify the primary lesion when metastases to other organs are observed, as EHE may come from either tissue and metastasize to the other or originate from more than one primary organ.…”

Section: Discussionmentioning

confidence: 99%

A Rare Case of Pulmonary Epithelioid Hemangioendothelioma Presenting with Skin Metastasis

Shin

Roh

et al. 2016

Arch Plast Surg

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Epithelioid hemangioendothelioma (EHE) is a well-differentiated and rare vascular tumor. Systemic metastases are uncommon. Herein, we present a patient with skin metastasis of pulmonary EHE (PEH) that was treated by wide excision. A 76-year-old male was evaluated due to pulmonary thromboembolism and a solitary pulmonary nodule. A biopsy was performed and pathological examination of the mass confirmed EHE. No metastasis was observed. The patient returned to care approximately two years later due to a painful nodule in the right lower leg. A skin biopsy showed metastatic EHE from the lung. We used a safety margin of 1 cm based on clinical experience, because no prior case had been reported regarding the resection margin appropriate for primary cutaneous EHE and skin metastases of PEH. At four months after surgery, the patient recovered without complications or recurrence. Skin metastasis of PEH is extremely rare, and only two cases have been reported in the literature. In this case, we report a rare case of PEH with histologically diagnosed skin metastasis that was successfully treated by curative resection. It is expected that this case report will provide a helpful contribution to the extant data regarding PEH metastases.

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“…72 Epithelioid haemangioendothelioma limited to skin is rare, and typically presents as a solitary lesion with variable clinical appearances, including an erythematous nodule, papule or plaque, hyperkeratosis, or a non-healing ulcer. 57,[73][74][75][76] Cutaneous EHE has a significantly more favourable outcome than EHE arising at other locations. Metastatic disease is very rare, 57,74,77,78 and complete excision is adequate treatment in the majority of cases.…”

Section: Epithelioid Haemangioendotheliomamentioning

confidence: 99%

Advances in the clinicopathological and molecular classification of cutaneous mesenchymal neoplasms

Costigan

Doyle

2016

Histopathology

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In recent years, there have been several important refinements in the classification of cutaneous mesenchymal neoplasms, including the description of new tumour types, along with the identification of novel and recurrent molecular genetic findings. In addition to providing new insights into tumour biology, many of these advances have had significant clinical consequences with regard to diagnostics, management, and prognostication. Newly described entities include pseudomyogenic haemangioendothelioma, haemosiderotic fibrolipomatous tumour, and fibroblastic connective tissue naevus, which are reviewed in the context of the principal differential diagnoses and significant clinical implications. Genetic characterization of several soft tissue tumour types that occur in the skin has resulted in the identification of diagnostically useful markers: ALK gene rearrangement with corresponding ALK protein expression by immunohistochemistry in epithelioid fibrous histiocytoma; the WWTR1-CAMTA1 fusion gene with CAMTA1 protein expression in epithelioid haemangioendothelioma; MYC amplification and overexpression in radiation-associated angiosarcoma; and EWSR1 gene rearrangement in cutaneous myoepithelial tumours. Finally, the classification of intradermal smooth muscle tumours and unclassified/pleomorphic dermal sarcoma has been refined, resulting in both improved classification and improved prognostication. Many of the tumour types listed above are encountered not only by specialist dermatopathologists, but also by practising general surgical pathologists, and this review should therefore provide a widely applicable update on the histological and molecular classification of cutaneous mesenchymal neoplasms, along with the appropriate use of ancillary diagnostic tests, in particular immunohistochemistry, in the evaluation of such lesions and their histological mimics.

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Cutaneous Epithelioid Hemangioendothelioma on the Sole of a Child

Cited by 9 publications

References 34 publications

Clinical characteristics of epithelioid hemangioendothelioma: a single-center retrospective study

Clinical characteristics of epithelioid hemangioendothelioma: a single-center retrospective study

A Rare Case of Pulmonary Epithelioid Hemangioendothelioma Presenting with Skin Metastasis

Advances in the clinicopathological and molecular classification of cutaneous mesenchymal neoplasms

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