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. 2026 Mar 6;18:20363613261433351. doi: 10.1177/20363613261433351

Primary epithelioid angiosarcoma of the vulva: A rare tumor at an unusual site

Elizabeta Miovska 1, Jasmina Dobrevska 1, Katerina Nikoloska 2, Bisera Nikolovska 1,✉
PMCID: PMC12966541  PMID: 41800210

Abstract

Epithelioid angiosarcoma of the vulva is an exceptionally rare and highly aggressive malignancy originating from endothelial cells. Its etiology remains unclear, though risk factors such as prior radiotherapy and chronic lymphedema have been identified. We report a case of a 44-year-old woman with no significant medical history who presented with a rapidly enlarging, foul-smelling mass on the right labia majora. The tumor measured 30 cm and exhibited central necrosis, ulceration, and bleeding. Radical tumor resection and right inguinal lymphadenectomy were performed, followed by reconstruction using a keystone design perforator island flap and a V-Y advancement flap. Histopathology confirmed a high-grade epithelioid angiosarcoma, positive for ERG, CD31, CD34, podoplanin, EMA, and a Ki-67 proliferation index of 60%. Further treatment consisted of adjuvant radiotherapy (61 Gy) and doxorubicin-based chemotherapy. At one-year follow-up, the patient remained free of recurrence or metastasis. Primary epithelioid angiosarcoma of the vulva is extremely rare, particularly in patients without known risk factors. Management involves radical surgical resection with adjuvant radiotherapy and chemotherapy as indicated. Reporting such cases is essential to enhance understanding and guide evidence-based management of these challenging neoplasms.

Keywords: rare tumors, angiosarcoma, keystone design perforator island flap, epitheloid angiosarcoma, gynecologic malignancy, vulvar epitheloid angiosarcoma

Introduction

Angiosarcomas are uncommon, aggressive malignant neoplasms that arise from endothelial cells of blood and lymphatic vessels. They represent about 2% of all soft tissue sarcomas, have locally aggressive behavior, and frequently metastasize. These tumors commonly occur in the head and neck—scalp in particular—the chest, extremities, trunk, liver, heart, bone, and spleen.1–4

It is a sporadic tumor in the female genital tract with a reported incidence of less than 1%. Among this subset of patients, primary vulvar angiosarcomas are very uncommon entities, having been described in only a handful of patients.5,6

The symptoms are generally nonspecific and mimic benign or other malignant vulvar disorders, making clinical diagnosis difficult to establish.6,7 Histopathological assessment with immunohistochemical staining, such as CD31, CD34, and ERG, is required for tumor diagnosis.8–10

Because of its rarity at this location, there is no evidence-based treatment, and available recommendations for treatment are usually derived from angiosarcomas occurring at other sites.2,5

Presentation

Patient history

Case: A 44-year-old woman, with a parity of 2, and with no significant past medical history, presented with an enlarging vulvar mass. She initially presented with a lesion on her right labia majora that she had noticed approximately 1 year ago, which did not increase in size until 2 months before presentation, when it began to grow rapidly and provoked discomfort when walking (Figure 1). The patient was referred to computed tomography (CT), magnetic resonance imaging (MRI), fine-needle aspiration (FNA), and core biopsies as a standard tumor work-up. Three months following her initial appointment, she came back for a follow-up visit, and the tumor was ten times bigger (Figure 2).

Figure 1.

Figure 1.

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Initial presentation of the tumor.

Figure 2.

Figure 2.

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Follow-up examination after 3 months.

Clinical examination

During the clinical examination, a large, exophytic vulvar mass approximately 30 cm in its greatest diameter was observed on the vulva, involving the right labia majora and minora, vaginal introitus, and mons pubis to the left labia majora. The lesion showed central ulceration with extensive necrosis and was actively bleeding. There was a significant foul-smelling discharge containing blood and necrotic tissue. The size and location of the mass impaired the patient’s mobility, affecting her ability to walk and perform daily activities. On palpation, the mass was firm, non-tender, and fixed to its base. Ipsilateral inguinal lymphadenopathy was noted, while no palpable contralateral inguinal lymph nodes were detected. On vaginal examination, the mass displaced the right vaginal wall but did not invade it. There was no involvement of the urethra or rectum.

Work-up

Preoperative MR imaging revealed invasion of the vagina, the rectus abdominis aponeurosis, and surrounding soft tissues (Figure 3). Cytological examination of the FNA was classified as group 5, strongly suggestive of malignant anaplastic sarcoma, although the tissue of origin could not be definitively determined. Core biopsies failed to provide definitive results despite two attempts due to large areas of necrosis. Because of the rapidly growing lesion and tumor’s large size, active bleeding, and foul smell, we chose not to delay treatment for further diagnostic procedures and proceeded directly with surgery, based on a working diagnosis of sarcoma.

Figure 3.

Figure 3.

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Preoperative contrast MRI of the pelvis. (a) Sagittal plane and (b) axial plane.

Surgical management

Radical tumor resection and a right inguinal lymphadenectomy were planned using a multidisciplinary surgical approach (a gynecologist and a plastic surgeon). A keystone design perforator island flap (KDPIF) with omega modification for pubic region reconstruction and a pedicled anterolateral thigh (ALT) flap for perineal, vulvar, and vaginal reconstruction were part of the reconstructive plan (Figure 4, photo a).

Figure 4.

Figure 4.

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(a) Preoperative planning; (b) anterior view; (c) frog leg view (tumor excision and right inguinal lymphadenectomy).

Surgical treatment included wide local excision of the tumor (3 cm margins), which included resection of the right labia majora and minora, partial resection of the left labia majora, mons pubis, aponeurosis of the rectus abdominis, periosteum of the pubic symphysis, and right lateral wall of the vagina (Figure 4 photos b and c). Intraoperatively, gross pathological vascularity was noted, which contributed to both preoperative and intraoperative bleeding issues. Right inguinal lymph node dissection was performed in view of the presence of clinically enlarged lymph nodes and the absence of a definitive diagnosis preoperatively. Intraoperatively, the plan for soft tissue reconstruction was modified after the tumor excision. The vaginal wall was reconstructed with direct suture, obviating the need for a pedicled ALT flap. The soft tissue defect measured 550 cm2 and was reconstructed using 2 perforator-based fasciocutaneous flaps: vulvar reconstruction was performed using a V-Y advancement flap from the right medial inner thigh. A large keystone flap was advanced to cover the defect in the pubic area (Figure 5).

Figure 5.

Figure 5.

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Reconstruction of the defect with KDPIF omega modification and V-Y advancement flap. (a) Anterior view; (b) frog leg view.

Histopathological findings

The specimen weighed 3580 grams, the tumor measured 24.5 x 23.5 x 21 cm, and the surface showed skin ulceration measuring 9 x 6.5 cm. The surgical margins on fixed material were: 1.1 cm-1.4 cm on lateral, upper, and lower resection margins, 0.5 cm from the nearest medial resection margin (vaginal wall), and the deep resection margin was 1 mm (periosteum of pubic bones), signifying extensive local invasion.

Microscopy revealed a malignant mesenchymal neoplasm, partially ulcerated and covered by stratified squamous epithelium, infiltrating the papillary and reticular dermis with dilated, congested vessels. The non-encapsulated, hypercellular lesion comprised polygonal to spindle-shaped cells with eosinophilic cytoplasm, vesicular nuclei, and prominent nucleoli. Rare, atypical cells with hyperchromatic nuclei and areas of solid, diffuse growth with focal vascular channel formation were identified.

Immunohistochemical staining was positive for ERG, CD31, CD34, Podoplanin, and EMA, and negative for SMA, Desmin, S-100, CD68, AE1/AE3, and HHV-8. The Ki-67 proliferation index was 60% in hotspots, and the mitotic index was 6-7 mitoses per 10 high-power fields.

The specimen from the lymphadenectomy revealed 4 lymph nodes with diameters of 1.1 cm to 1.9 cm, and microscopic analysis of the nodes showed reactive changes without a neoplastic substrate.

Adjuvant treatment

Post-operatively, the patient’s recovery was satisfactory, and good healing of the reconstructed site was observed. Two months after surgery, adjuvant treatment with chemo and radiotherapy was conducted: the patient received 61 Gy of radiotherapy and doxorubicin monotherapy (6 cycles 70mg/m2 q 21 days). The patient had excellent compliance with oncological treatment with no adverse side effects reported.

Follow-up

The flaps used for reconstruction are durable and structurally intact after radiotherapy (Figure 6). No local recurrence or metastasis observed clinically and radiographically at 1-year follow-up: magnetic resonance imaging of the pelvis at 1 year after surgery showed no radiological signs of local recurrence, and PET-CT showed no evidence of distant metastasis (Figure 7). Regular follow-up of the patient is continued every 3 months.

Figure 6.

Figure 6.

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One year follow-up. (a) Anterior view; (b) frog leg view.

Figure 7.

Figure 7.

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Pelvic MRI with contrast one year post-operatively. (a) Sagittal plane; (b) axial plane.

Discussion

Wagner et al. evaluated the incidence of angiosarcoma, demographics, and extent of disease at presentation in all reported patients with a new diagnosis of angiosarcoma in the USA from 2001 to 2020 (n = 19,289). Visceral angiosarcomas were reported in 24.4% (n = 4,701), of which 2.2% (n = 102) were found in the female reproductive system. 11 An evidence-based historical review by Sheinis et al. and Stolnicu et al. found that the publication on cases of vulvar angiosarcoma was so scant that it is likely to be one of the rarest types of cancer at this location.7,12 While exact risk factors remain incompletely described, several associations have been reported, including chronic lymphedema, a history of radiotherapy to the area, exposure to foreign material or substances, prior trauma or surgery and specific hereditary cancer syndromes.3,13,14 Of particular interest, our case had no obvious risk factors.

Most published case reports of vulvar angiosarcoma are associated with previous radiotherapy or other risk factors (Table 1). To date, two documented cases of primary vulvar angiosarcoma in healthy patients have been reported, and the epithelioid subtype has been histologically and immunohistochemically confirmed in only one of these cases.7,15 Sheinis et al. described a case of primary multifocal angiosarcoma of the vulva without reporting the relevant medical history, and in the report of Lafever et al. previous surgical interventions were noted (incision and drainage, and hysterectomy, and bilateral salpingo-oophorectomy) that may represent a potential risk factor.12,14,16

Table 1.

Reported cases of vulvar angiosarcoma (2003-2021).

Author, year Age (yrs) Risk factors Location on the vulva Tumor size (cm) Histologic type Immunohistochemistry Treatment Recurence/metastasis Follow up Outcome
Our case 44 None Right labia majora extending to mons pubis 24.5 x 23.5 x 21 cm Epithelioid angiosarcoma ERG+, CD31+, CD34+, Podoplanin+, EMA+; Ki-67 60%, SMA−, Desmin−, S-100−, CD68−, AE1/AE3−, HHV-8− Radical excision; Adjuvant doxorubicin and radiotherapy None 12 NED
Stolnicu et al. 2021 7 43 Primarly misdiagnosed as angiofibroma Left labia majora Primary lesion: fragments 1.5–4.2 cm; recurrence lesion: 6 cm Poorly differentiated angiosarcoma CD31+, Vimentin+, EMA+; Desmin−, S100−, Myogenin−, SMA− Surgical excision; referred to oncology; adjuvant details NR Local recurrence after 3 years 36 AWD
Sheinis et al. 2016 12 64 NR Vulva (not specified) 2.2 cm Multifocal angiosarcoma CD31+, D2-40+, ERG+; HHV-8−, TFE-3− Vulvectomy + bilateral inguinal lymphadenectomy; paclitaxel, nab-paclitaxel,radiation, gemcitabine 1st recurrence: 3 weeks (vulva + left obturator LN); 2nd recurrence: 4 months; metastasis to bone; 1 of 5 inguinal LN positive 24 AWD
Das & Sonowal 2021 15 40 None Mons pubis to left labia majora 10 × 10 × 4 cm Epithelioid angiosarcoma CD31+, Vimentin+, EMA+; Desmin−, S100−, Myogenin−, SMA− Surgical excision NR NR NR
Lafever et al. 2014 16 67 Prior melanoma; two incision and drainage procedures on vulvar lesions prior to diagnosis; total abdominal hysterectomy with bilateral salpingo-oophorectomy Multifocal: mons, bilateral labia majora Multiple: 0.5, 1.8, 2.5 cm High-grade angiosarcoma CD31+, CD34+ Paclitaxel adriamycin, bevacizumab, valproic acid, radiation Disseminated at diagnosis (liver, pancreas, kidney) <12 DOD
Yost et al. 2017 4 90 Post-radiation + lymphedema Labia majora extending to mons pubis 20 × 10cm Grade 2 epithelioid angiosarcoma CD31+, ERG+, Vimentin+; CD34− Declined (hospice) No treatment given NR AWD
Mayer et al. 2021 17 65 Post-radiation Labia majora, left mid-lateral predominant 2 cm nodules, bilateral involvement High-grade angiosarcoma CD31+, C-MYC+, Ki67 elevated; Pancytokeratin− Radical bilateral vulvectomy NR 13 NED
Guirguis et al. 2007 18 74 Post-radiation Mons pubis NR Epithelioid angiosarcoma NR Surgical resection Recurrence at 6 months; metastatic disease >6 AWD
Kondi-Pafiti et al. 2003 19 76 Post-radiation Mons pubis, with extension to labia majora 14 × 12 cm High-grade angiosarcoma Vimentin +; Factor VIII + Extensive surgical excision None at 1 year 12 NED
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Abbreviations: yrs = years; cm = centimeters; NR = Not reported; DOD = Died of disease; AWD = Alive with disease; NED = No evidence of disease.

Epithelioid angiosarcomas characteristically express endothelial markers including CD31, ERG, CD34 (often variable), Factor VIII, and Podoplanin (D2-40 in subsets), with high proliferative indices such as elevated Ki-67 (>50% hotspots) and increased mitoses (typically >5/10 HPF). They lack melanocytic (S-100, HMB-45, Melan-A), epithelial (AE1/AE3, usually EMA), smooth muscle (SMA, Desmin), histiocytic (CD68), and HHV-8 markers, distinguishing them from melanoma, carcinoma, leiomyosarcoma, histiocytic tumors, and Kaposi sarcoma. This immunoprofile, combined with epithelioid morphology, confirms the diagnosis of high-grade epithelioid angiosarcoma.9,20

Gessi et al. detected HHV-8 in angiosarcoma tissue via in situ hybridization and PCR, suggesting an association with angiosarcoma. 21 However, Schmid and Zietz’s subsequent study of 40 independent angiosarcomas, including vulvar cases, demonstrated that HHV-8 was not detectable in the tumor tissue. 22

The rapid progression observed in aggressive epithelioid angiosarcoma is driven by abnormal endothelial proliferation, dysregulated angiogenesis, activation of oncogenic pathways, and a highly infiltrative phenotype, all of which were evident histologically and morphologically in our case. Together, these findings define a highly aggressive neoplasm with strong potential for recurrence or metastasis and a poor prognosis. 23

Radical surgical excision is the most appropriate approach for the complete removal of these aggressive and infiltrative tumors, allowing for local disease control and prevention of recurrence. Achieving R0 resection significantly reduces the risk of tumor regrowth and improves survival. 24

Sarcoma resection creates large soft tissue defects due to wide oncologic margins. Reconstruction prioritizes stable coverage, protection of vital structures, preservation of function, aesthetics, low donor morbidity, and support for adjuvant therapy. In pelvic cases, it focuses on urinary/sexual function and quality of life using versatile, low-morbidity perforator flaps (single or multiple) for challenging defects while retaining pliability.25,26

The keystone perforator island flap is increasingly used in sarcoma reconstruction for its reliable vascularity, simplicity, and versatility, while the V-Y island flap from the thigh is a well-established technique for vulvar reconstruction following oncologic resection or extensive defects, offering reliable vascularity based on perforator vessels, minimal donor site morbidity, and good aesthetic and functional outcomes.27–31

Sarcomas most often metastasize through hematogenous routes; however, certain histological subtypes, including rhabdomyosarcoma, epithelioid sarcoma, clear cell sarcoma, and angiosarcoma, tend to spread via the lymphatic system. 24 Involvement of regional lymph nodes has been reported in 10% to 40% of angiosarcoma cases. Chan et al. documented lymph node metastases in approximately 23% of 150 patients with analyzed angiosarcoma. 32

In our case, a right-sided lymphadenectomy was performed because of palpable lymph nodes and their proximity to the primary tumor. The decision to proceed with lymphadenectomy was influenced by the tumor’s large size, necrotic and bleeding characteristics, and closeness to regional lymphatic pathways. This approach aligns with the current understanding that, in high-risk, large, or necrotic tumors, lymphadenectomy enhances local control and provides valuable staging information to guide adjuvant therapy decisions. 24

Adjuvant chemotherapy combined with radiotherapy is used to target microscopic tumor foci that cannot be eliminated by surgical resection alone.4,12,23 Most commonly used drugs include taxanes and anthracyclines such as paclitaxel and doxorubicin. Paclitaxel is the standard first-line chemotherapy for epithelioid angiosarcoma, including vulvar forms, in both localized and metastatic disease. Doxorubicin may be used as an alternative or in combination regimens. 33 Combination regimens include carboplatin-paclitaxel-pembrolizumab for advanced disease, doxorubicin-cyclophosphamide-paclitaxel for aggressive cases, and gemcitabine-docetaxel when paclitaxel is contraindicated.2,34–38

Adjuvant radiation therapy for angiosarcoma typically involves delivering a total dose of 60-70 Gy in daily fractions of 2.0-2.5 Gy over 6 to 7 weeks, using modern techniques such as intensity-modulated radiation therapy (IMRT) to maximize local control while reducing toxicity. This method is especially recommended for high-grade tumors, positive or close margins, and large or deeply invasive lesions, often combined with chemotherapy to improve outcomes. 39

There are no published formal clinical practice guidelines from major organizations (NCCN, ESMO, ASCO) specifically addressing neoadjuvant therapy for angiosarcoma. Heinhuis et al.’s systematic review shows neoadjuvant chemotherapy downsizes tumors, enables negative margins, and reduces surgical morbidity across sites. 40 Though unreported for vulvar angiosarcoma, neoadjuvant radiotherapy is reasonable in advanced cases for sarcoma and vulvar carcinoma guidelines to downstage the disease and improve outcomes. 41

For patients who are not candidates for surgical resection, multimodal systemic therapy comprising chemotherapy, radiotherapy, and/or immunotherapy may be used as a palliative measure. Nevertheless, the evidence supporting these non-surgical approaches is limited, particularly for vulvar angiosarcoma, and such treatment decisions should be individualized based on patient performance status and disease characteristics.41–43

The optimal management of angiosarcomas currently involves radical surgical resection with wide margins, complex reconstruction, and adjuvant chemoradiotherapy.33,39

The survival statistics for epithelioid angiosarcoma of the vulva are poor, with over 50% of patients being deceased within 12 months of diagnosis, and the estimated 5-year overall survival is less than 30%. High mitotic rate and grade, as well as positive surgical margins, are associated with increased recurrence and mortality. These underscore the aggressive nature of this malignancy and the importance of early diagnosis and intensive multimodal therapy.5,17 In our case, on a one-year follow-up, the patient showed no evidence of locoregional recurrence or distant metastasis.

Conclusion

This case report presents the successful treatment of an uncommon and aggressive vulvar epithelioid angiosarcoma using radical surgery, complex reconstruction, and adjuvant chemoradiotherapy. The report highlights key aspects of managing rare and aggressive tumors, including the importance of early detection despite diagnostic challenges, the necessity of aggressive surgical procedures with appropriate reconstruction, the need for multimodal treatment, and the value of a collaborative approach to enhance patient outcomes. Further research and reporting of similar cases are essential for developing evidence-based guidelines for managing these tumors.

Acknowledgments

The authors acknowledge that an abstract of this work was previously published in the abstract book of the 3rd International Case Report Congress, organized by the Macedonian Medical Association (International Case Report Congress, Skopje, 4–7 April 2025) and is available in the official abstract book of the 3rd International Case Report Congress by the Macedonian Medical Association (https://icrc.mld.mk/wp-content/uploads/2025/04/ICRC-abstrakt-kniga-1.pdf).

Footnotes

Author contributions: Bisera Nikolovska MD PhD and Katerina Nikoloska MD PhD were attending physicians and senior authors (investigation and supervision), approved the manuscript for submition, Elizabeta Miovska MD was responsible for conceptualisation, data curation and writing of the original draft, Jasmina Dobrevska MD provided clinical materials, patient images and consent aquisition.

Funding: The authors received no financial support for the research, authorship, and/or publication of this article.

The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

ORCID iDs

Elizabeta Miovska https://orcid.org/0009-0003-6324-9559

Jasmina Dobrevska https://orcid.org/0009-0004-8315-6138

Katerina Nikoloska https://orcid.org/0000-0003-1367-2546

Bisera Nikolovska https://orcid.org/0000-0002-9594-2601

Ethical considerations

The University Clinic for Plastic and Reconstructive Surgery in Skopje does not require ethical approval for reporting individual case reports. This case report is based solely on previously collected, non-identifiable information and did not involve any research involving human subjects. Ethical approval was therefore not required for this case report, in accordance with national legislation in North Macedonia and institutional policy at Ss. Cyril and Methodius University in Skopje.

Consent for publication

The patient provided informed consent, and a signed record is maintained at our institution. Informed consent was obtained from the patient, and a signed record is kept at the University clinic for plastic and reconstructive surgery in Skopje.

Data Availability Statement

All relevant data supporting the findings of this case report are included within the article. Additional information regarding this case may be available from the corresponding author upon reasonable request, subject to patient confidentiality. The patient’s identity has been anonymized.*

References

  • 1.Kim WJ, Kim HK. Current understanding of angiosarcoma: disease biology and evolving treatment. Arch Craniofac Surg 2023; 24(5): 203–210. 10.7181/acfs.2023.00409 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 2.Cao J, Wang J, He C, et al. Angiosarcoma: a review of diagnosis and current treatment. Am J Cancer Res 2019; 9(11): 2303–2313. PMID: 31815036; PMCID: PMC6895451. [PMC free article] [PubMed] [Google Scholar]
  • 3.Koch M, Nielsen GP, Yoon SS. Malignant tumors of blood vessels: Angiosarcomas, hemangioendotheliomas, and hemangioperictyomas. J Surg Oncol 2008; 97(4): 321–329. 10.1002/jso.20973 [DOI] [PubMed] [Google Scholar]
  • 4.Yost S, Bradish J, Grossheim L, et al. Epithelioid angiosarcoma of the vulva: A case report. Gynecol Oncol Rep 2017; 21: 91–93. 10.1016/j.gore.2017.07.010 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 5.Kruse AJ, Sep S, Slangen BFM, et al. Angiosarcomas of primary gynecologic origin: A clinicopathologic review and quantitative analysis of survival. International Journal of Gynecological Cancer 2014; 24(1): 4–12. 10.1097/IGC.0000000000000020 [DOI] [PubMed] [Google Scholar]
  • 6.Suchak R, Thway K, Zelger B, et al. Primary cutaneous epithelioid angiosarcoma: a clinicopathologic study of 13 cases of a rare neoplasm occurring outside the setting of conventional angiosarcomas and with predilection for the limbs. Am J Surg Pathol 2011; 35(1): 60–69. Erratum in: Am J Surg Pathol. 2011 Apr; 35(4):619. PMID: 21164288. 10.1097/PAS.0b013e3181fee872 [DOI] [PubMed] [Google Scholar]
  • 7.Stolnicu S, Podoleanu C, Orban I, et al. Primary angiosarcoma of the vulva: Report of a case misdiagnosed as a benign tumor. Polish Journal of Pathology 2021; 72(2): 167–169. 10.5114/pjp.2021.109519 [DOI] [PubMed] [Google Scholar]
  • 8.Miettinen M, Wang Z, Sarlomo-Rikala M, et al. ERG Expression in Epithelioid Sarcoma. American Journal of Surgical Pathology 2013; 37(10): 1580–1585. 10.1097/pas.0b013e31828de23a [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 9.Hart J, Mandavilli S. Epithelioid angiosarcoma: a brief diagnostic review and differential diagnosis. Arch Pathol Lab Med 2011; 135(2): 268–272. PMID: 21284449. 10.5858/135.2.268 [DOI] [PubMed] [Google Scholar]
  • 10.Sulliva HC, Edgar MA, Cohen C, et al. The utility of ERG, CD31 and CD34 in the cytological diagnosis of angiosarcoma: An analysis of 25 cases. J Clin Pathol 2015; 68(1): 44–50. 10.1136/jclinpath-2014-202629 [DOI] [PubMed] [Google Scholar]
  • 11.Wagner MJ, Ravi V, Schaub SK, et al. Incidence and Presenting Characteristics of Angiosarcoma in the US, 2001-2020. JAMA Netw Open 2024; 7(4): e246235. PMID: 38607625; PMCID: PMC11015348. 10.1001/jamanetworkopen.2024.6235 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 12.Sheinis M, Cesari M, Selk A. Angiosarcoma of the Vulva: A Case Report. J Low Genit Tract Dis 2016; 20(1): e6–e7. PMID: 26704338. 10.1097/LGT.0000000000000171 [DOI] [PubMed] [Google Scholar]
  • 13.Roohani S, Rotermund T, Ehret F, et al. Angiosarcoma: clinical outcomes and prognostic factors, a single-center analysis. J Cancer Res Clin Oncol 2024; 150(6): 326. PMID: 38914779; PMCID: PMC11196347. 10.1007/s00432-024-05835-x [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 14.Young RJ, Brown NJ, Reed MW, et al. Angiosarcoma. Lancet Oncol 2010; 11(10): 983–991. 10.1016/S1470-2045(10)70023-1. Epub 2010 May 25. PMID: 20537949. [DOI] [PubMed] [Google Scholar]
  • 15.Das P, Sonowal R. Large epithelioid vulvar angiosarcoma in postmenopausal woman: a rare case report. SAS J Surg 2021; 7(3): 137–140. 10.36347/sasjs.2021.v07i03.007 [DOI] [Google Scholar]
  • 16.Lafever A, Tyler EM, Stockdale CK. Angiosarcoma of the vulva. Proceedings in Obstetrics and Gynecology 2014; 4(2): 1–4. 10.17077/2154-4751.1254 [DOI] [Google Scholar]
  • 17.Meyer M, Smith ES, Leitao M, Jr. Angiosarcoma of the vulva following radiation for colorectal cancer. Gynecol Oncol Rep 2021; 36: 100711. PMID: 33598522; PMCID: PMC7868731. 10.1016/j.gore.2021.100711 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 18.Guirguis A, Kanbour-Shakir A, Kelley J. Epithelioid angiosarcoma of the mons after chemoradiation for vulvar cancer. Int J Gynecol Pathol 2007; 26(3): 265–268. PMID: 17581409. 10.1097/01.pgp.0000250145.67462.c9 [DOI] [PubMed] [Google Scholar]
  • 19.Kondi-Pafiti A, Kairi-Vassilatou E, Spanidou-Carvouni H, et al. Vascular tumors of the female genital tract: a clinicopathological study of nine cases. Eur J Gynaecol Oncol 2003; 24(1): 48–50. PMID: 12691317. [PubMed] [Google Scholar]
  • 20.Chokoeva AA, Tchernev G, Cardoso JC, et al. Vulvar sarcomas: Short guideline for histopathological recognition and clinical management. Part 2. Int J Immunopathol Pharmacol 2015; 28(2): 178–186. 10.1177/0394632015575977. Epub 2015 Mar 26. PMID: 25816393. [DOI] [PubMed] [Google Scholar]
  • 21.Gessi M, Cattani P, Maggiano N, et al. Demonstration of human herpesvirus 8 in a case of primary vaginal epithelioid angiosarcoma by in situ hybridization, electron microscopy, and polymerase chain reaction. Diagn Mol Pathol 2002; 11(3): 146–151. PMID: 12218453. 10.1097/00019606-200209000-00004 [DOI] [PubMed] [Google Scholar]
  • 22.Schmid H, Zietz C. Human herpesvirus 8 and angiosarcoma: analysis of 40 cases and review of the literature. Pathology 2005; 37(4): 284–287. 10.1080/00313020500169495.PMID:16194826 [DOI] [PubMed] [Google Scholar]
  • 23.Luong HTT, Vercammen S, de Marco A, et al. Angiosarcoma: a systematic review of biomarkers in diagnosis, prognosis, and therapeutic strategies. Front Oncol 2025; 15: 1623327. PMID: 40666093; PMCID: PMC12259444. 10.3389/fonc.2025.1623327 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 24.Dunaj P, Żukowska E, Czarnecka AM, et al. Lymphadenectomy in the treatment of sarcomas - indications and technique. Oncol Rev 2024; 18: 1413734. PMID: 39737200; PMCID: PMC11683405. 10.3389/or.2024.1413734 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 25.Miyamoto S, Arikawa M, Kagaya Y. The use of lower abdominal perforator flaps in soft-tissue reconstruction after sarcoma resection. Microsurgery 2020; 40(3): 353–360. 10.1002/micr.30539. Epub 2019 Dec 13. PMID: 31833597. [DOI] [PubMed] [Google Scholar]
  • 26.Stiles ZE, Lohman RF, Mann GN. Plastic Surgery Reconstruction of Sarcoma Resection Defects: Form and Function. Surg Clin North Am 2022; 102(4): 583–599. 10.1016/j.suc.2022.04.008. Epub 2022 Jun 24. PMID: 35952689. [DOI] [PubMed] [Google Scholar]
  • 27.Behan FC. The Keystone Design Perforator Island Flap in reconstructive surgery. ANZ J Surg 2003; 73(3): 112–120. PMID: 12608972. 10.1046/j.1445-2197.2003.02638.x [DOI] [PubMed] [Google Scholar]
  • 28.Yan M, Rose PS, Houdek MT, et al. Outcomes of the keystone perforator island flap for oncologic reconstruction of the back. J Surg Oncol 2021; 124(7): 1002–1007. 10.1002/jso.26629. Epub 2021 Jul 29. PMID: 34324204. [DOI] [PubMed] [Google Scholar]
  • 29.Lee Y, Lee S, Lee D, et al. Vulvar Reconstruction Using Keystone Flaps Based on the Perforators of Three Arteries. Arch Plast Surg 2022; 49(6): 724–728. PMID: 36523907; PMCID: PMC9747278. 10.1055/s-0042-1756294 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 30.Peiretti M, Corvetto E, Candotti G, et al. New Keystone flap application in vulvo-perineal reconstructive surgery: A case series. Gynecol Oncol Rep 2019; 30: 100505. PMID: 31687469; PMCID: PMC6819813. 10.1016/j.gore.2019.100505 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 31.Hand LC, Maas TM, Baka N, et al. Utilizing V-Y fasciocutaneous advancement flaps for vulvar reconstruction. Gynecol Oncol Rep 2018; 26: 24–28. PMID: 30186930; PMCID: PMC6122364. 10.1016/j.gore.2018.08.007 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 32.Chan JY, Tan GF, Yeong J, et al. Clinical implications of systemic and local immune responses in human angiosarcoma. NPJ Precis Oncol 2021; 5(1): 11. PMID: 33580206; PMCID: PMC7881182. 10.1038/s41698-021-00150-x [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 33.Ishida Y, Otsuka A, Kabashima K. Cutaneous angiosarcoma: update on biology and latest treatment. Curr Opin Oncol 2018; 30(2): 107–112. PMID: 29194075; PMCID: PMC5815647. 10.1097/CCO.0000000000000427 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 34.Bramwell VH, Anderson D, Charette ML, et al. Doxorubicin-based chemotherapy for the palliative treatment of adult patients with locally advanced or metastatic soft tissue sarcoma. Cochrane Database Syst Rev 2003; 2003(3): CD003293. PMID: 12917960; PMCID: PMC7144828. 10.1002/14651858.CD003293 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 35.Constantinidou A, Sauve N, Stacchiotti S, et al. Evaluation of the use and efficacy of (neo)adjuvant chemotherapy in angiosarcoma: a multicentre study. ESMO Open 2020; 5(4): e000787. PMID: 32847839; PMCID: PMC7451279. 10.1136/esmoopen-2020-000787 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 36.Lewcun JA, Pameijer C, Kass R, et al. Doxorubicin, paclitaxel, and cisplatin based chemotherapy for the treatment of angiosarcoma: Two case reports. Int J Surg Case Rep 2020; 68: 83–87. 10.1016/j.ijscr.2020.02.036. Epub 2020 Feb 21. PMID: 32120283; PMCID: PMC7052479. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 37.Penel N, Italiano A, Ray-Coquard I, et al. Metastatic angiosarcomas: doxorubicin-based regimens, weekly paclitaxel and metastasectomy significantly improve the outcome. Ann Oncol 2012; 23(2): 517–523. 10.1093/annonc/mdr138. Epub 2011 May 12. PMID: 21566149. [DOI] [PubMed] [Google Scholar]
  • 38.Jones RL, Ravi V, Brohl AS, et al. Efficacy and Safety of TRC105 Plus Pazopanib vs Pazopanib Alone for Treatment of Patients With Advanced Angiosarcoma: A Randomized Clinical Trial. JAMA Oncol 2022; 8(5): 740–747. PMID: 35357396; PMCID: PMC89. 10.1001/jamaoncol.2021.3547 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 39.Smrke A, Hamm J, Karvat A, et al. A retrospective review of 145 patients with angiosarcoma: Radiation therapy, extent of resection and chemotherapy are important predictors of survival. Mol Clin Oncol 2020; 13(2): 179–185. 10.3892/mco.2020.2055. Epub 2020 Jun 2. PMID: 32714543; PMCID: PMC7366222. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 40.Heinhuis KM, IJzerman NS, van der Graaf WTA, et al. Neoadjuvant Systemic Treatment of Primary Angiosarcoma. Cancers (Basel) 2020; 12(8): 2251. PMID: 32806524; PMCID: PMC7464310. 10.3390/cancers12082251 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 41.Roohani S, Wiltink LM, Kaul D, et al. Update on Dosing and Fractionation for Neoadjuvant Radiotherapy for Localized Soft Tissue Sarcoma. Curr Treat Options Oncol 2024; 25(4): 543–555. 10.1007/s11864-024-01188-2. Epub 2024 Mar 13. PMID: 38478330; PMCID: PMC10997691. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 42.Sotelo J, Sharma M, Schultz S. Post-radiation Angiosarcoma of the Vagina: A Case Report and Literature Review. Cureus 2024; 16(10): e72475. PMID: 39600772; PMCID: PMC11594438. 10.7759/cureus.72475 [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 43.Florou V, Rosenberg AE, Wieder E, et al. Angiosarcoma patients treated with immune checkpoint inhibitors: a case series of seven patients from a single institution. J Immunother Cancer 2019; 7(1): 213. Erratum in: J Immunother Cancer. 2019 Nov 6; 7(1):285. doi: 10.1186/s40425-019-0792-9. PMID: 31395100; PMCID: PMC6686562. 10.1186/s40425-019-0689-7 [DOI] [PMC free article] [PubMed] [Google Scholar]

Associated Data

This section collects any data citations, data availability statements, or supplementary materials included in this article.

Data Availability Statement

All relevant data supporting the findings of this case report are included within the article. Additional information regarding this case may be available from the corresponding author upon reasonable request, subject to patient confidentiality. The patient’s identity has been anonymized.*

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