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Vulvar epithelioid hemangioendothelioma
Gynecologic Oncology 105 (2007) 539 – 541 www.elsevier.com/locate/ygyno
Case Report
Vulvar epithelioid hemangioendothelioma Benedito Borges da Silva ⁎, Pedro Vitor Lopes-Costa, Ana Maria Furtado-Veloso, Rafael Soares Borges Department of Gynecology, Federal University of Piauí, Teresina, Piauí, Brazil Received 19 November 2006 Available online 15 February 2007
Abstract Background. Epithelioid hemangioendothelioma (EH) is a rare vascular tumor whose localization in the vulva is extremely rare. Case. The present case report refers to a 19-year-old Brazilian woman admitted to hospital with an extensive lesion of granulomatous appearance on the right labium majus. Following confirmation of the diagnosis of epithelioid hemangioendothelioma, the only treatment consisted of hemivulvectomy and right inguinal lymphadenectomy. Patient was lost to follow-up for 8 years after recovery from surgery. She reappeared 4 years ago and is now being followed-up. There is no sign of recurrence of the disease. Conclusion. EH is a lesion of unpredictable malignancy whose localization in the vulva is extremely rare. The first therapeutic option is surgical resection. © 2007 Elsevier Inc. All rights reserved. Keywords: Vulva; Cancer; Epithelioid hemangioendothelioma; Vulvar neoplasia; Sarcoma
Introduction Epithelioid hemangioendotheliomas (EH) are rare vascular tumors, the majority of which originate in soft tissues, lungs or liver [1–8]. Their localization in the vulva is extremely rare. To the best of our knowledge, only one case of vulvar EH, specifically localized in the clitoris, has been reported in the literature [1] to which we would like to add this case of an extensive epithelioid hemangioendothelioma of the labia majora. Case report In March 1994, a 19-year-old Brazilian woman (gravida 0, para 0) was admitted to this hospital complaining of a vulvar lesion that had appeared approximately 2 years previously, following her first sexual encounter. Physical examination revealed an extensive, swollen, granulomatous lesion affecting
⁎ Corresponding author. Avenida Elias João Tajra, 1260, Apt. 600, Bairro Jockey Club, 64.049-300 Teresina, Piauí, Brazil. Fax: +55 86 3215 0470. E-mail address: [email protected] (B.B. da Silva). 0090-8258/$ - see front matter © 2007 Elsevier Inc. All rights reserved. doi:10.1016/j.ygyno.2007.01.022
the entire right labium majus and part of the mons pubis, marked in places by folds and with small ulcerations resembling granulomatous vulvitis in its chronic form of pseudoelephantiasis (Fig. 1). Palpable ipsilateral inguinal lymph nodes, the largest measuring around 1.3 cm, were found, whereas contralateral inguinal lymph nodes were not palpable. Physical examination revealed an apparently normal vagina and cervix. Cervical smear was negative for malignancy and colposcopy was normal. Histology and immunohistochemistry of the biopsy sample resulted in confirmation of vulvar epithelioid hemangioendothelioma. Chest and abdominal computed tomography scans detected no abnormalities. The patient was submitted to hemivulvectomy with right inguinal lymphadenectomy. Gross examination of the surgical specimen revealed a segment of the vulva measuring 9 × 5 × 3 cm, covered by a grossly granular, brownish-blackened epiderm with diverse fistulous orifices. At the sections, the surface of the lesion was glossy whitish-yellow with focal areas of necrosis and hemorrhage and with fistulous orifices of up to 1.0 cm in size. Histology detected neoplasia characterized by cords and nests of large polygonal cells, the majority containing a moderate amount of eosinophilic, somewhat glassy appearing cytoplasm, and oval nuclei with bland chromatin and incon-
540
B.B. da Silva et al. / Gynecologic Oncology 105 (2007) 539–541
Fig. 1. Extensive hypertrophied, edematous and fibrotic vulvar lesion originating in the right labium majus. The lesion is shown at the start of resection.
spicuous nucleoli. Prominent small blood vessels were found within and around the tumor cells. Immunohistochemistry detected positivity of the tumor cells for factor VIII-related antigen and CD34, which, together with the histological findings, led to confirmation of the diagnosis of epithelioid hemangioendothelioma. No metastasis was found in the resected inguinal lymph nodes, only an inflammatory reaction. All resected borders of the surgical specimen were clear. The resected area healed well and the final cosmetic result was satisfactory. After the wound had healed, the patient failed to return for follow-up. Eight years later, the patient returned having given birth to two children. She has now been followed-up for 4 years and there are no signs of recurrence of the disease. Discussion Epithelioid hemangioendothelioma is a rare vascular tumor of unknown etiology, usually originating in soft tissues, lungs or liver, although it has been reported at virtually any site, including the brain and bone [1–8]. Up to the present, only one case of vulval EH has been reported in the literature [1]. In the present case, taking the macroscopic characteristics of the lesion and the history of the patient into consideration, the first suspected diagnosis was donovanosis in its rarest form, pseudoelephantiasis [9]. This suspicion was, however, refuted following histological and immunohistochemical examination of the lesion, which stained positive for factor VIII-related antigen and CD34, leading to confirmation of the diagnosis of epithelioid hemangioendothelioma.
Epithelioid hemangioendothelioma may appear in several forms, usually as a slightly painful mass in either superficial or deep soft tissue. The liver is the soft tissue most commonly affected by EH. The presence of a solitary tumor constitutes an initial stage of the disease; however, the majority of cases consist of multiple hepatic tumors [7,8]. The first therapeutic option is hepatic resection or liver transplantation in the case of diffuse liver disease [7,8]. In a series of 137 cases of EH of the liver, Makhlouf et al. [8] concluded that the prognosis was much more favorable than that of other hepatic malignancies despite a metastatic rate of 27%. Prolonged survival (5– 28 years) was reported following resection or liver transplantation [8]. Of the 60 patients followed-up for a prolonged period, 43% (26/60) survived ≥ 5 years, whereas 54% (13/24) of the patients who did not receive treatment were still alive at the final follow-up after 18 years. Tumor regression was reported in one untreated patient at the 18-year follow-up, who presented with an enlarged liver, a phenomenon that could be explained by the dense fibrotic stroma cutting off the circulation to the neoplastic cells [8]. Therefore, patients with EH of the liver may survive for various years without any specific treatment, while others may proceed rapidly to a fatal outcome [4,7,8]. The role of adjuvant chemotherapy with hepatic intraarterial 5-fluorouracil, radiotherapy, and/or treatment with interferon-α2 in cases of EH of the liver remains unclear [7,8] since the small number of treated cases and the unpredictable evolution of EH does not permit conclusions to be drawn [4,7,8]. EH of the liver may metastasize to any part of the body; however, the most frequent sites are the lungs and abdominal lymph nodes [4,7,8]. Interestingly, metastasis spread during surgery does not correlate with survival and is not considered a contraindication for surgery [7,8]. Therefore, surgical resection is the therapy of choice for EH and in the present case consisted of hemivulvectomy and unilateral lymphadenectomy. None of the lymph nodes examined contained metastatic cells and the contralateral inguinal lymph nodes were not examined. Bearing in mind the unpredictability of malignancy and the evolution of EH, therapy was shown to be effective in this case since the patient was treated 12 years ago and is currently in follow-up with no signs of recurrence of the disease. References [1] Strayer SA, Yum MN, Sutton GP. Epithelioid hemangioendothelioma of the clitoris: a case report with immunohistochemical and ultrastructural findings. Int J Gynecol Pathol 1992;11(3):234–9. [2] Faust J, Schmidt M, Eysel P, Wanitschke R. Epithelioid hemangioendothelioma of the spine. Med Klin 2001;96(12):740–4. [3] Ramer MA, Lumerman H, Kopp W, Fisher KS, Cohen SA. Epithelioid hemangioendothelioma of the maxilla: case report and review of the literature. Periodontal Clin Invest 2001;23(1):31–5. [4] Uchimura K, Nakamuta M, Osoegawa M, Takeaki S, Nishi H, Iwamoto H, et al. Hepatic epithelioid hemangioendothelioma. J Clin Gastroenterol 2001;32(5):431–4. [5] Sakamoto N, Adachi S, Monzawa S, Hamanaka A, Takada Y, Hunada Y, et al. High resolution CT findings of pulmonary epithelioid hemangioendothelioma: unusual manifestations in 2 cases. J Thorac Imaging 2005;20 (3):236–8.
B.B. da Silva et al. / Gynecologic Oncology 105 (2007) 539–541 [6] Lee JC, Lee BJ, Wang SG, Kim HW. Epithelioid haemangioendothelioma in the parapharyngeal space. J Laryngol Otol 2006;120(6):505–7. [7] Lauffer JM, Zimmermann A, Krahenbuhl L, Triller J, Baer HU. Epithelioid hemangioendothelioma of the liver. A rare hepatic tumor. Cancer 1996;78 (11):2318–27.
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[8] Makhlouf HR, Ishak KG, Goodman ZD. Epithelioid hemangioendothelioma of the liver: a clinicopathologic study of 137 cases. Cancer 1999;85 (3):562–82. [9] Silva BB, Costa PV, Dias HK, Batista SM. Vulvar donovanosis as pseudoelephantiasis. Rev Assoc Med Bras 2006;52(1):11.
Case Report
Vulvar epithelioid hemangioendothelioma Benedito Borges da Silva ⁎, Pedro Vitor Lopes-Costa, Ana Maria Furtado-Veloso, Rafael Soares Borges Department of Gynecology, Federal University of Piauí, Teresina, Piauí, Brazil Received 19 November 2006 Available online 15 February 2007
Abstract Background. Epithelioid hemangioendothelioma (EH) is a rare vascular tumor whose localization in the vulva is extremely rare. Case. The present case report refers to a 19-year-old Brazilian woman admitted to hospital with an extensive lesion of granulomatous appearance on the right labium majus. Following confirmation of the diagnosis of epithelioid hemangioendothelioma, the only treatment consisted of hemivulvectomy and right inguinal lymphadenectomy. Patient was lost to follow-up for 8 years after recovery from surgery. She reappeared 4 years ago and is now being followed-up. There is no sign of recurrence of the disease. Conclusion. EH is a lesion of unpredictable malignancy whose localization in the vulva is extremely rare. The first therapeutic option is surgical resection. © 2007 Elsevier Inc. All rights reserved. Keywords: Vulva; Cancer; Epithelioid hemangioendothelioma; Vulvar neoplasia; Sarcoma
Introduction Epithelioid hemangioendotheliomas (EH) are rare vascular tumors, the majority of which originate in soft tissues, lungs or liver [1–8]. Their localization in the vulva is extremely rare. To the best of our knowledge, only one case of vulvar EH, specifically localized in the clitoris, has been reported in the literature [1] to which we would like to add this case of an extensive epithelioid hemangioendothelioma of the labia majora. Case report In March 1994, a 19-year-old Brazilian woman (gravida 0, para 0) was admitted to this hospital complaining of a vulvar lesion that had appeared approximately 2 years previously, following her first sexual encounter. Physical examination revealed an extensive, swollen, granulomatous lesion affecting
⁎ Corresponding author. Avenida Elias João Tajra, 1260, Apt. 600, Bairro Jockey Club, 64.049-300 Teresina, Piauí, Brazil. Fax: +55 86 3215 0470. E-mail address: [email protected] (B.B. da Silva). 0090-8258/$ - see front matter © 2007 Elsevier Inc. All rights reserved. doi:10.1016/j.ygyno.2007.01.022
the entire right labium majus and part of the mons pubis, marked in places by folds and with small ulcerations resembling granulomatous vulvitis in its chronic form of pseudoelephantiasis (Fig. 1). Palpable ipsilateral inguinal lymph nodes, the largest measuring around 1.3 cm, were found, whereas contralateral inguinal lymph nodes were not palpable. Physical examination revealed an apparently normal vagina and cervix. Cervical smear was negative for malignancy and colposcopy was normal. Histology and immunohistochemistry of the biopsy sample resulted in confirmation of vulvar epithelioid hemangioendothelioma. Chest and abdominal computed tomography scans detected no abnormalities. The patient was submitted to hemivulvectomy with right inguinal lymphadenectomy. Gross examination of the surgical specimen revealed a segment of the vulva measuring 9 × 5 × 3 cm, covered by a grossly granular, brownish-blackened epiderm with diverse fistulous orifices. At the sections, the surface of the lesion was glossy whitish-yellow with focal areas of necrosis and hemorrhage and with fistulous orifices of up to 1.0 cm in size. Histology detected neoplasia characterized by cords and nests of large polygonal cells, the majority containing a moderate amount of eosinophilic, somewhat glassy appearing cytoplasm, and oval nuclei with bland chromatin and incon-
540
B.B. da Silva et al. / Gynecologic Oncology 105 (2007) 539–541
Fig. 1. Extensive hypertrophied, edematous and fibrotic vulvar lesion originating in the right labium majus. The lesion is shown at the start of resection.
spicuous nucleoli. Prominent small blood vessels were found within and around the tumor cells. Immunohistochemistry detected positivity of the tumor cells for factor VIII-related antigen and CD34, which, together with the histological findings, led to confirmation of the diagnosis of epithelioid hemangioendothelioma. No metastasis was found in the resected inguinal lymph nodes, only an inflammatory reaction. All resected borders of the surgical specimen were clear. The resected area healed well and the final cosmetic result was satisfactory. After the wound had healed, the patient failed to return for follow-up. Eight years later, the patient returned having given birth to two children. She has now been followed-up for 4 years and there are no signs of recurrence of the disease. Discussion Epithelioid hemangioendothelioma is a rare vascular tumor of unknown etiology, usually originating in soft tissues, lungs or liver, although it has been reported at virtually any site, including the brain and bone [1–8]. Up to the present, only one case of vulval EH has been reported in the literature [1]. In the present case, taking the macroscopic characteristics of the lesion and the history of the patient into consideration, the first suspected diagnosis was donovanosis in its rarest form, pseudoelephantiasis [9]. This suspicion was, however, refuted following histological and immunohistochemical examination of the lesion, which stained positive for factor VIII-related antigen and CD34, leading to confirmation of the diagnosis of epithelioid hemangioendothelioma.
Epithelioid hemangioendothelioma may appear in several forms, usually as a slightly painful mass in either superficial or deep soft tissue. The liver is the soft tissue most commonly affected by EH. The presence of a solitary tumor constitutes an initial stage of the disease; however, the majority of cases consist of multiple hepatic tumors [7,8]. The first therapeutic option is hepatic resection or liver transplantation in the case of diffuse liver disease [7,8]. In a series of 137 cases of EH of the liver, Makhlouf et al. [8] concluded that the prognosis was much more favorable than that of other hepatic malignancies despite a metastatic rate of 27%. Prolonged survival (5– 28 years) was reported following resection or liver transplantation [8]. Of the 60 patients followed-up for a prolonged period, 43% (26/60) survived ≥ 5 years, whereas 54% (13/24) of the patients who did not receive treatment were still alive at the final follow-up after 18 years. Tumor regression was reported in one untreated patient at the 18-year follow-up, who presented with an enlarged liver, a phenomenon that could be explained by the dense fibrotic stroma cutting off the circulation to the neoplastic cells [8]. Therefore, patients with EH of the liver may survive for various years without any specific treatment, while others may proceed rapidly to a fatal outcome [4,7,8]. The role of adjuvant chemotherapy with hepatic intraarterial 5-fluorouracil, radiotherapy, and/or treatment with interferon-α2 in cases of EH of the liver remains unclear [7,8] since the small number of treated cases and the unpredictable evolution of EH does not permit conclusions to be drawn [4,7,8]. EH of the liver may metastasize to any part of the body; however, the most frequent sites are the lungs and abdominal lymph nodes [4,7,8]. Interestingly, metastasis spread during surgery does not correlate with survival and is not considered a contraindication for surgery [7,8]. Therefore, surgical resection is the therapy of choice for EH and in the present case consisted of hemivulvectomy and unilateral lymphadenectomy. None of the lymph nodes examined contained metastatic cells and the contralateral inguinal lymph nodes were not examined. Bearing in mind the unpredictability of malignancy and the evolution of EH, therapy was shown to be effective in this case since the patient was treated 12 years ago and is currently in follow-up with no signs of recurrence of the disease. References [1] Strayer SA, Yum MN, Sutton GP. Epithelioid hemangioendothelioma of the clitoris: a case report with immunohistochemical and ultrastructural findings. Int J Gynecol Pathol 1992;11(3):234–9. [2] Faust J, Schmidt M, Eysel P, Wanitschke R. Epithelioid hemangioendothelioma of the spine. Med Klin 2001;96(12):740–4. [3] Ramer MA, Lumerman H, Kopp W, Fisher KS, Cohen SA. Epithelioid hemangioendothelioma of the maxilla: case report and review of the literature. Periodontal Clin Invest 2001;23(1):31–5. [4] Uchimura K, Nakamuta M, Osoegawa M, Takeaki S, Nishi H, Iwamoto H, et al. Hepatic epithelioid hemangioendothelioma. J Clin Gastroenterol 2001;32(5):431–4. [5] Sakamoto N, Adachi S, Monzawa S, Hamanaka A, Takada Y, Hunada Y, et al. High resolution CT findings of pulmonary epithelioid hemangioendothelioma: unusual manifestations in 2 cases. J Thorac Imaging 2005;20 (3):236–8.
B.B. da Silva et al. / Gynecologic Oncology 105 (2007) 539–541 [6] Lee JC, Lee BJ, Wang SG, Kim HW. Epithelioid haemangioendothelioma in the parapharyngeal space. J Laryngol Otol 2006;120(6):505–7. [7] Lauffer JM, Zimmermann A, Krahenbuhl L, Triller J, Baer HU. Epithelioid hemangioendothelioma of the liver. A rare hepatic tumor. Cancer 1996;78 (11):2318–27.
541
[8] Makhlouf HR, Ishak KG, Goodman ZD. Epithelioid hemangioendothelioma of the liver: a clinicopathologic study of 137 cases. Cancer 1999;85 (3):562–82. [9] Silva BB, Costa PV, Dias HK, Batista SM. Vulvar donovanosis as pseudoelephantiasis. Rev Assoc Med Bras 2006;52(1):11.