Background: To report a rare case of retiform hemangioendothelioma (RH)
originated from the vulva and review the relative references. Case: A
38-year-old woman was admitted to our hospital with a vulvar subcutaneous nodule
(diameter about 2 cm) for 2 years. The clinical examination findings, laboratory
test results, including complete blood count and liver function test results were
normal. The B mode ultrasound revealed that a hypoechoic nodule with a clear
boundary was observed on the right side of the vulva. The size was measured to be
23 mm
Retiform hemangioendothelioma (RH) is a rare intermediate vasogenic tumor, which was first described in 1994 [1], with less than 40 cases reported in the worldwide [2]. Different from cutaneous angiosarcoma, the clinical manifestations RH are intermediate between a benign hemangioma and a high-grade angiosarcoma, therefore, the term “intermediary malignant tumor” was defined. Women in middle-aged are affected more commonly [3]. The limbs and trunk are the commonly involved organs of RH, follow by penis, scalp, jejunum and external genital area [4, 5, 6]. Distant metastasis is rare, however, local recurrence occurs in about 50% cases [7]. Herein, we report a case of RH in the vulvar area, and discuss the immunophenotypic features and treatment methods of RH in the vulva.
A 38-year-old woman with a medical history notable for caesarean section for 14
years, a uterine myoma (diameter about 6 cm) for 5 years, and a vulvar
subcutaneous nodule with a diameter about 2 cm for 2 years prior to presentation.
Physical examination revealed that a mass with approximately 2 cm in diameter on
the right vulva. The margin of the mass was well-defined, the texture was in a
medium quality and fair mobilizable activity. There were no tenderness or
ulceration on the surface. No abnormality was found in the laboratory tests
including carbohydrate antigen 125, cervical screening for human papillomavirus
(high-risk type) and liquid-based cervical cytology. Transvaginal ultrasound
showed that a hypoechoic area of 57 mm
Ultrasound image of the vulvar nodule: B mode ultrasound showed clear boundary and blood flow signals.
The characteristics of tumor histopathology. (A) Elongated,
arborizing, thin-walled vessels (H&E staining,
The etiology of RH is still unknown [7]. Although several cases suggested its association with human herpesvirus-8, nonepidermal malignant tumors, previous radiation treatment and prior lymphangioma lymphedema, the exact associations have not been explicitly established [8, 9, 10, 11].
RH has no specific clinical, laboratory or radiographic characteristics. However, in this case, B mode ultrasound showed clear boundary and blood flow signals, which may provide more information available for the diagnosis of RH. The diagnosis of RH is completely depended on the histopathological findings. The typical RH tumor tissue is characterized by a network of elongated and arborizing vessels, which similar to the normal testicular structure. The inner wall of the blood vessel is arranged with a monolayer cells, which have uniform morphology, hyperchromatic nuclei, sparse cytoplasm, and generally no atypia or nuclear mitosis [12, 13]. Immunohistochemical staining results are featured by positive factor VIII-related antigen, CD31, CD34 and D2-40, and negative or weakly positive SMA [2, 4, 14, 15, 16].
Surgical resection for early-stage RH is the most common treatment. However, the standard routine of the operation has not been determined. According to Emberger et al. [15], it was necessary to expand the surgical range to 3 cm out of the edge of the tumor, because the high local recurrence rate of RH. In this case, the expanded resection with only 1 cm extended cutting edge was performed due to the vulvar position. The patient has been monitored for 15 months after surgery, and no local recurrence has been found, suggesting that the feasibility of 1 cm resection of early-stage RH of the vulva.
In conclusion, RH of vulvar area is rather rare. The diagnosis of RH is clinically and radiologically challenging, and the pathology examination is necessary. Local expanded resection of tumor may be an effective method for the treatment of early-stage RH to reduce the risk of local recurrence.
Fli-1, Friend leukemia virus integration 1; RH, Retiform hemangioendothelioma; SMA, Smooth muscle actin.
HJW and ZCD were major contributors in writing the manuscript, XY performed the histological examination, HJW, ZCD and YY analyzed and interpreted the patient data. All authors read and approved the final manuscript.
All participants have given their consent for use of the medical data.
Thanks to all the peer reviewers for their opinions and suggestions.
This research received no external funding.
The authors declare no conflict of interest.