Primary Vulvar Lymphangioma Circumscriptum: A Case Report and Review of Literature Arif Kokcu, MD,1 Seher Sari, MD,1 and Mehmet Kefeli, MD2 Departments of 1Obstetrics and Gynecology and 2Pathology, Faculty of Medicine, University of Ondokuz Mayis, Samsun, Turkey
h Abstact To add a new case of primary vulvar lymphangioma circumscriptum to the literature and to review the current literature. Materials and Methods. We searched the PubMed/ MEDLINE databases for previous case reports using the key words ‘‘vulvar lymphangioma circumscriptum’’ and ‘‘primary vulvar lymphangioma circumscriptum.’’ Results. We found 21 cases of primary vulvar lymphangioma circumscriptum reported until 2013. Mean age was 32 years (range = 3Y76 y), and 62 % of the cases had been treated surgically. Conclusions. The treatment of primary vulvar lymphangioma circumscriptum is mainly surgery. Depending on the age of the patient and the extent of lesion, surgical treatment might be in the form of the local excision, labiectomy, or vulvectomy. h Objective.
Key Words: vulva, primary, lymphangioma, circumscriptum
L
ymphangiomas are rare benign proliferations of the lymphatic system. There are 3 types of lymphangioma: circumscriptum, cavernous, and cystic. Lymphangioma circumscriptum (LC) is considered either primary or congenital if it is present at birth or if it develops in early childhood or secondary or acquired if it is caused by impaired lymph flow [1]. However, the primary vulvar LC occurs mostly in adulthood. Secondary lymphangioma is
Reprint requests to: Arif Kokcu, MD, Department of Obstetrics and Gynecology, School of Medicine, University of Ondokuz Mayis, Kurupelit, 55139 Samsun, Turkey. E-mail: [email protected] No funding was received for this study. The authors have declared they have no conflicts of interest.
Ó 2014, American Society for Colposcopy and Cervical Pathology Journal of Lower Genital Tract Disease, Volume 19, Number 1, 2015, e1Ye5
also termed acquired lymphangioma and lymphangiectasis [2]. Lymphangioma circumscriptum was first described in 1878 by Fox and Fox, who called it lymphangiectodes; Malcolm Morris first used the term LC in 1889. Pathologic features were described by Noyes and Torok in 1890 and by Francis in 1893. Peachy et al. suggested the connection of the superficial vesicles with deep lymphatic cisterns [3, 4]. Vulvar involvement by lymphangioma is rare. To date, 21 cases of the primary vulvar LC have been reported in the literature [4Y22]. We describe a new case of the primary vulvar form and present a brief review of the literature. To the best of our knowledge, this is the 22nd case of primary vulvar LC.
CASE REPORT A 35-year-old woman presented with complaints of pain and swelling in her vulva persisting for the past 6 years. She had taken various drugs for her problem but all failed. Clinical examination revealed well-defined, multiple, grouped, dome-shaped papulovesicular lesions on both labia major, with partly uneven and partly verrucous surface (see Figure 1). There was watery secretion on the vulvar surface. The lesions were not tender and showed diffuse induration. Both the labia minora and vagina were normal. Inguinal lymph nodes were absent. Results of the remaining genital and general examination were normal. She had no history of surgery, radiotherapy, gastrointestinal disease, sexually transmitted disease, genital ulcer, trauma, tuberculosis, pelvic infection, or filariasis. Results of pelvic and transvaginal ultrasonography (US) and Pap smear tests were normal. Color Doppler ultrasound showed no abnormal findings in the bilateral
Copyright © 2014 American Society for Colposcopy and Cervical Pathology. Unauthorized reproduction of this article is prohibited.
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KOKCU ET AL.
DISCUSSION
Figure 1. Gross appearance of bilateral vulvar lymphangioma circumscriptum.
iliac artery and venous systems. Pelvic magnetic resonance imaging did not demonstrate any enlargement or cystic dilatation in lymphatics along the pelvic-parailiac regions. Tuberculosis was ruled out by acid-resistant bacilli test, erythrocyte sedimentation rate, and chest radiograph. A nontreponemal test for syphilis was nonreactive. She underwent biopsy of vulvar lesions, and vulvar LC was diagnosed. Bilateral major labiectomy was done with liberal use of electrocautery at the depth of excision. Primary closure of skin was done (see Figure 2). Histopathologic examination revealed multiple dilated vascular channels in papillary dermis, lined by a thin wall, consisting only of endothelial cells. The overlying epidermis was partly hyperkeratotic, with thinning of stratum malpighii (see Figure 3). Diagnosis was confirmed as vulvar LC. The postoperative course was uneventful. Follow-up of 16 weeks revealed no recurrence (see Figure 4).
The etiologic factors for primary vulvar LC are not clear. Primary lesions seem to mature as patients mature and their external genital organs develop, until the lesions finally become apparent in adulthood [18, 23]. Although primary LC is reported to have developed at birth and in early childhood, 86% (19/22) of the reported primary vulvar LC cases were in the adult period. The youngest age of presentation of primary vulvar LC, to date, was 3 years, the oldest was 76 years, and the mean age was 32 years (see Table 1). Primary vulvar LC is a developmental defect of lymphatics in the deep and subcutaneous layers of the vulva. Lymphangiogenic growth factors, vascular endothelial growth factors C and D, and their receptors on lymphatic endothelial cells, vascular endothelial growth factor 3, may play a role in mechanisms controlling the development of LC [24, 25]. It is supposed to arise from local, closed, sequestrated lymphatic cisterns in the reticular dermis. They have muscular lining and connect with superficial lymphatics via dilated channels. These muscular lymphatic cisterns pulsate and transmit pressure, secondary to which dilatation of lymphatics occurs in dermal papillae visible as vesicles externally [26]. The proposed etiology of secondary vulvar LC is the architectural disruption of previously normal channels, leading to secondary vulvar LC by the sequestration and further dilatation of previously normal lymphatics [4, 19]. The predisposing conditions for secondary vulvar LC are radical surgery and/or radiation therapy for cervical neoplasia, infections (acute cellulitis, filariasis, sexually transmitted diseases, genital tuberculosis, lymph node tuberculosis, erysipelas, and lymphogranuloma venereum), Crohn disease, primary dysplastic angiopathy, bilateral varicose veins in the lower extremities, dermopathy from
Figure 2. Appearance of bilateral major labiectomy. A, Marked excision boundaries of lesion. B, Excised labia majora. C, Closed labiectomy field.
Copyright © 2014 American Society for Colposcopy and Cervical Pathology. Unauthorized reproduction of this article is prohibited.
Primary Vulvar Lymphangioma Circumscriptum
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Figure 3. Thin-walled, ectatic lymphatic channels in the papillary dermis. There is mild perivascular lymphocytic inflammatory infiltrate (hematoxylin-eosin stain; A, 25; B, 100; C, 200; D, 400).
penicillamine or corticosteroids, surgical trauma, keloids, scleroderma, and rhabdomyosarcoma [2, 19, 27]. Radiotherapy is generally being accused of being a causative factor for the secondary form of vulvar LC when there is a previous history of radiation treatment because of pelvic malignancies [15]. Because none of the etiologic factors mentioned above are shown in the present case, primary vulvar LC was diagnosed (see Figure 1). To date, in the English-language medical literature, primary LC of the vulva has been reported only in 21 patients, excluding the one reported in this article.
Figure 4. Appearance of vulva after 20 weeks of operation.
Clinically, LC is characterized by translucent vesicles, which may be scattered or grouped like frog-spawn. Rarely, the lesions can have a firm hyperkeratotic appearance. Vulvar LC can be asymptomatic, pruritic, burning, or painful. It may cause distressing symptoms of itching, pain, oozing of serous fluid, tendency to infections, psychosexual dysfunction, and cosmetic disfigurement [14, 15, 28]. Complications associated with vulval LC include cellulitis and psychosexual dysfunction. Follow-up of patients with lymphedema is recommended because transformation to lymphangiosarcoma may occur in the edematous limbs. Two cases of squamous cell carcinoma and lymphangiosarcoma arising within the LC have been reported [2]. Differential diagnosis of vulvar LC includes herpes zoster, molluscum contagiosum, genital warts, tuberculosis verrucosacutis (lupus verrucosus), leiomyoma, cellular angiofibroma, angiomyofibroblastoma, and aggressive angiomyxoma. If associated with significant hyperkeratosis, the clinical presentation may mimic condyloma acuminata. Biopsy specimen of the lesion forms the mainstay for diagnosis [17, 20, 24, 26]. There is no standard treatment of choice described for the management of vulvar LC. Depending on the type and extent of LC, the treatment options include procedures such as observation, abrasive modalities (carbon dioxide laser, liquid nitrogen, electrocoagulation, or sclerosing therapy), superficial radiotherapy, and surgical
Copyright © 2014 American Society for Colposcopy and Cervical Pathology. Unauthorized reproduction of this article is prohibited.
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KOKCU ET AL.
Table 1. Cases of Primary Vulvar Lymphangioma Circumscriptum Reported Until 2013 and the Applied Treatments No.
Date
Authors
Age, y
1 2
1981 1989
14 42
Radical vulvectomy Partial vulvectomy
3 4 5 6 7
1991
Bauer et al. [5] Abu-Hamad et al. [6] Johnson et al. [7]
32 35 20 76 20
Local Excision Vulvectomy Vulvectomy Patient refused treatment Local Excision (2 times)
8
1998
28
9
1999
24
Patient refused the treatment Observation
10 11 12 13 14 15
2001 2002 2006 2006 2007 2008
48 30 35 8 37 17
Simple vulvectomy Radical wide local excision Radical vulvectomy Sclerosing therapy Radiotherapy Major labiectomy
16 17 18
2008 2009
18 72 3
Major labiectomy Vulvectomy Observation
36 44 24
Vulvectomy Conservative treatment Physiotherapy
35
Major labiectomy
19 20 21 22
1992 1995 1995
Murugan [8] Cecchi et al. [9] Short and Peacock [10] Nishi [11] Gordon and Kaufman [12] Sah et al. [13] Vlastos et al. [14] Roy et al. [4] Ahn et al. [15] Yildiz et al. [16] Ghaemmaghami et al. [17]
Dikmen et al. [18] Aggarwal et al. [19] 2010 Basak et al. [20] 2012 Shah et al. [21] 2013 Chattranukulchai et al. [22] Present case Kokcu et al.
Treatment
excision (local excision, simple or extended vulvectomy) [2, 4, 16, 20]. Treating superficial lesions with methods such as carbon dioxide laser therapy and superficial radiotherapy has resulted in a short-term cure. Abrasive modalities have advantages of outpatient procedure, regional anesthesia, and repetition in case of recurrence [26]. These treatment modalities were applied to but rather secondary cases (only 2 of the primary cases). External radiotherapy was administered in only 1 case of primary vulvar LC and led to a surprisingly good response [16]. However, radiotherapy may cause premature ovarian failure and, very rarely, secondary malignancies. The disadvantage of abrasive modalities is a high recurrence rate (55.6% within an average period of 4.5 mo). Other complaints are oozing, lymphorrhea, and recurrent erysipelas. Hence, a close follow-up and early treatment of recurrence are a must for this modality [4]. The most preferred treatment for both the primary and secondary types is surgical excision. In surgical excision, the deep-feeding lymphatic cisterns should be removed to prevent recurrence [17]. Surgical management of vulvar lymphangioma offers a more permanent solution. However, even with the best treatment option,
recurrence is common. One should take adequate precautions to excise lymphatic cisterns in deep subcutaneous tissue for better results [26]. Fourteen of 22 cases of primary vulvar LC, including the one reported in this article, were treated surgically. Excisional surgical procedures for the treatment of primary vulvar LC varied from local excision to radical vulvectomy (see Table 1). In the present case, after bilateral major labiectomy, vulvar pain of the patient has disappeared as well, and the appearance of the vulva after 20 weeks of operation was normal (see Figures 2 and 3). The extent of the lesion also affects the surgical outcome and recurrence rates. The recurrence rate after the surgical procedure was 23.1% on variable follow-up ranging from 6 to 81 months [26]. The aim of this case report was to highlight an unusual and extremely rare case of vulvar disease. Primary vulvar LC poses a diagnostic challenge to treating gynecologists because of various clinical mimics. When diagnosed, even with the best treatment options, recurrences are common. Depending on the age of the patient and the extent of the lesion, local excision, major labiectomy, and vulvectomy are the preferred modes of treatment for primary forms especially.
REFERENCES 1. Shetty V, Venkatesh S. Acquired lymphangioma circumscriptum of the vulva. Int J Gynaecol Obstet 2012;117:190. 2. Amouri M, Masmoudi A, Boudaya S, Amouri A, Ben Ali I, Bouassida S, et al. Acquired lymphangioma circumscriptum of the vulva. Dermatol Online J 2007;13:10. 3. Esquivias Go´mez JI, Miranda-Romero A, Cuadrado Valle´s C, Bajo del Pozo C, Sa´nchez Sambucety P, Martı´nez Ferna´ndez M, et al. Lymphangioma circumscriptum of the vulva. Cutis 2001;67:229Y32. 4. Roy KK, Agarwal R, Agarwal S, Kumar S, Malhotra N, Gopendru N. Recurrent vulval congenital lymphangioma circumscriptumVa case report and literature review. Int J Gynecol Cancer 2006;16:930Y4. 5. Bauer BS, Kernahan DA, Hugo NE. Lymphangioma circumscriptumVa clinicopathological review. Ann Plast Surg 1981;7:318Y26. 6. Abu-Hamad A, Provencher D, Ganjei P, Penalver M. Lymphangioma circumscriptum of the vulva: case report and review of the literature. Obstet Gynecol 1989;73:496Y9. Review. 7. Johnson TL, Kennedy AW, Segal GH. Lymphangioma circumscriptum of the vulva. A report of two cases. J Reprod Med 1991;36:808Y12. 8. Murugan S. Lymphangioma circumscriptum of the vulva. J Indian Med Assoc 1993;91:77.
Copyright © 2014 American Society for Colposcopy and Cervical Pathology. Unauthorized reproduction of this article is prohibited.
Primary Vulvar Lymphangioma Circumscriptum
9. Cecchi R, Bartoli L, Brunetti L, Pavesi M, Giomi A. Lymphangioma circumscriptum of the vulva of late onset. Acta Derm Venereol 1995;75:79Y80. 10. Short S, Peacock C. A newly described possible complication of lymphangioma circumscriptum. Clin Oncol 1995; 7:136Y7. 11. Nishi T. Lymphangioma of the labia minora with deep lymphatic involvement. Br J Obstet Gynaecol 1998;105: 926Y7. 12. Gordon D, Kaufman R. How best can one diagnose and manage vulvar lymphangioma circumscriptum? J Low Genit Tract Dis 1999;3:218Y20. 13. Sah SP, Yadav R, Rani S. Lymphangioma circumscriptum of the vulva mimicking genital wart: a case report and review of literature. J Obstet Gynaecol Res 2001;27: 293Y6. 14. Vlastos AT, Malpica A, Follen M. Lymphangioma circumscriptum of the vulva: a review of literature. Obstet Gynecol 2003;101:946Y54. 15. Ahn SJ, Chang SE, Choi JH, Moon KC, Koh JK, Kim DY. A case of unresectable lymphangioma circumscriptum of the vulva successfully treated with OK-432 in childhood. J Am Acad Dermatol 2006;55(suppl 5):S106Y7. 16. Yildiz F, Atahan IL, Ozyar E, Karcaaltincaba M, Cengiz M, Ozyigit G, et al. Radiotherapy in congenital vulvar lymphangioma circumscriptum. Int J Gynecol Cancer 2008; 18:556Y9. 17. Ghaemmaghami F, Karimi Zarchi M, Mousavi A. Major labiaectomy as surgical management of vulvar lymphangioma circumscriptum: three cases and a review of the literature. Arch Gynecol Obstet 2008;278:57Y60. 18. Dikmen Y, Cosan Terek M, Akman L, Solmaz U, Zekiog˘lu O, Akalin T. Congenital vulvar lymphangioma circumscriptum. Int J Clin Oncol 2008;13:458Y60.
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19. Aggarwal K, Gupta S, Jain VK, Marwah N. Congenital lymphangioma circumscriptum of the vulva. Indian Pediatr 2009;46:428Y9. 20. Basak S, De A, Bag T. Surgery as the treatment of choice in vulvar lymphangioma circumscriptum: case report and review of other management options. Eur J Obstet Gynecol Reprod Biol 2010;152:225Y6. 21. Shah TN, Shekokar S, Venkatesh S, Santosh KV, Santosh KV. Lymphangioma circumscriptum of the vulva: a rare case report. Eur J Obstet Gynecol Reprod Biol 2012;165:131Y2. 22. Chattranukulchai P, Satitthummanid S, Puwanant S, Boonyaratavej S. Lymphangioma circumscriptum of the vulva. BMJ Case Rep 2013;2013. pii: bcr2013009297. doi:10.1136/ bcr-2013-009297. 23. Vlastos AT, Malpica A, Follen M. Lymphangioma circumscriptum of the vulva: a review of the literature. Obstet Gynecol 2003;101(5 Pt 1):946Y54. Review. 24. Go¨nu¨l M, Cakmak SK, Soylu S, Kilic¸ A, Gu¨ndu¨z H, Gu¨l U, et al. Lymphangioma circumscriptum of the vulva: a case report. Acta Derm Venereol 2009;89:180Y1. 25. Sultan A, Dadras SS, Bay JM, Teng NN. Prox-1, podoplanin and HPV staining assists in identification of lymphangioma circumscriptum of the vulva and discrimination from vulvar warts. Histopathology 2011;59:1274Y7. 26. Ghaemmaghami F, Karimi Zarchi M, Mousavi A. Surgical management of primary vulvar lymphangioma circumscriptum and postradiation: case series and review of literature. J Minim Invasive Gynecol 2008;15:205Y8. 27. Vignes S, Arrault M, Tre´vidic P. Surgical resection of vulva lymphoedema circumscriptum. J Plast Reconstr Aesthet Surg 2010;63:1883Y5. 28. Tulasi NR, John A, Chauhan I, Nagarajan V, Geetha G. Lymphangioma circumscriptum. Int J Gynecol Cancer 2004; 14:564Y6.
Copyright © 2014 American Society for Colposcopy and Cervical Pathology. Unauthorized reproduction of this article is prohibited.
h Abstact To add a new case of primary vulvar lymphangioma circumscriptum to the literature and to review the current literature. Materials and Methods. We searched the PubMed/ MEDLINE databases for previous case reports using the key words ‘‘vulvar lymphangioma circumscriptum’’ and ‘‘primary vulvar lymphangioma circumscriptum.’’ Results. We found 21 cases of primary vulvar lymphangioma circumscriptum reported until 2013. Mean age was 32 years (range = 3Y76 y), and 62 % of the cases had been treated surgically. Conclusions. The treatment of primary vulvar lymphangioma circumscriptum is mainly surgery. Depending on the age of the patient and the extent of lesion, surgical treatment might be in the form of the local excision, labiectomy, or vulvectomy. h Objective.
Key Words: vulva, primary, lymphangioma, circumscriptum
L
ymphangiomas are rare benign proliferations of the lymphatic system. There are 3 types of lymphangioma: circumscriptum, cavernous, and cystic. Lymphangioma circumscriptum (LC) is considered either primary or congenital if it is present at birth or if it develops in early childhood or secondary or acquired if it is caused by impaired lymph flow [1]. However, the primary vulvar LC occurs mostly in adulthood. Secondary lymphangioma is
Reprint requests to: Arif Kokcu, MD, Department of Obstetrics and Gynecology, School of Medicine, University of Ondokuz Mayis, Kurupelit, 55139 Samsun, Turkey. E-mail: [email protected] No funding was received for this study. The authors have declared they have no conflicts of interest.
Ó 2014, American Society for Colposcopy and Cervical Pathology Journal of Lower Genital Tract Disease, Volume 19, Number 1, 2015, e1Ye5
also termed acquired lymphangioma and lymphangiectasis [2]. Lymphangioma circumscriptum was first described in 1878 by Fox and Fox, who called it lymphangiectodes; Malcolm Morris first used the term LC in 1889. Pathologic features were described by Noyes and Torok in 1890 and by Francis in 1893. Peachy et al. suggested the connection of the superficial vesicles with deep lymphatic cisterns [3, 4]. Vulvar involvement by lymphangioma is rare. To date, 21 cases of the primary vulvar LC have been reported in the literature [4Y22]. We describe a new case of the primary vulvar form and present a brief review of the literature. To the best of our knowledge, this is the 22nd case of primary vulvar LC.
CASE REPORT A 35-year-old woman presented with complaints of pain and swelling in her vulva persisting for the past 6 years. She had taken various drugs for her problem but all failed. Clinical examination revealed well-defined, multiple, grouped, dome-shaped papulovesicular lesions on both labia major, with partly uneven and partly verrucous surface (see Figure 1). There was watery secretion on the vulvar surface. The lesions were not tender and showed diffuse induration. Both the labia minora and vagina were normal. Inguinal lymph nodes were absent. Results of the remaining genital and general examination were normal. She had no history of surgery, radiotherapy, gastrointestinal disease, sexually transmitted disease, genital ulcer, trauma, tuberculosis, pelvic infection, or filariasis. Results of pelvic and transvaginal ultrasonography (US) and Pap smear tests were normal. Color Doppler ultrasound showed no abnormal findings in the bilateral
Copyright © 2014 American Society for Colposcopy and Cervical Pathology. Unauthorized reproduction of this article is prohibited.
e2
&
KOKCU ET AL.
DISCUSSION
Figure 1. Gross appearance of bilateral vulvar lymphangioma circumscriptum.
iliac artery and venous systems. Pelvic magnetic resonance imaging did not demonstrate any enlargement or cystic dilatation in lymphatics along the pelvic-parailiac regions. Tuberculosis was ruled out by acid-resistant bacilli test, erythrocyte sedimentation rate, and chest radiograph. A nontreponemal test for syphilis was nonreactive. She underwent biopsy of vulvar lesions, and vulvar LC was diagnosed. Bilateral major labiectomy was done with liberal use of electrocautery at the depth of excision. Primary closure of skin was done (see Figure 2). Histopathologic examination revealed multiple dilated vascular channels in papillary dermis, lined by a thin wall, consisting only of endothelial cells. The overlying epidermis was partly hyperkeratotic, with thinning of stratum malpighii (see Figure 3). Diagnosis was confirmed as vulvar LC. The postoperative course was uneventful. Follow-up of 16 weeks revealed no recurrence (see Figure 4).
The etiologic factors for primary vulvar LC are not clear. Primary lesions seem to mature as patients mature and their external genital organs develop, until the lesions finally become apparent in adulthood [18, 23]. Although primary LC is reported to have developed at birth and in early childhood, 86% (19/22) of the reported primary vulvar LC cases were in the adult period. The youngest age of presentation of primary vulvar LC, to date, was 3 years, the oldest was 76 years, and the mean age was 32 years (see Table 1). Primary vulvar LC is a developmental defect of lymphatics in the deep and subcutaneous layers of the vulva. Lymphangiogenic growth factors, vascular endothelial growth factors C and D, and their receptors on lymphatic endothelial cells, vascular endothelial growth factor 3, may play a role in mechanisms controlling the development of LC [24, 25]. It is supposed to arise from local, closed, sequestrated lymphatic cisterns in the reticular dermis. They have muscular lining and connect with superficial lymphatics via dilated channels. These muscular lymphatic cisterns pulsate and transmit pressure, secondary to which dilatation of lymphatics occurs in dermal papillae visible as vesicles externally [26]. The proposed etiology of secondary vulvar LC is the architectural disruption of previously normal channels, leading to secondary vulvar LC by the sequestration and further dilatation of previously normal lymphatics [4, 19]. The predisposing conditions for secondary vulvar LC are radical surgery and/or radiation therapy for cervical neoplasia, infections (acute cellulitis, filariasis, sexually transmitted diseases, genital tuberculosis, lymph node tuberculosis, erysipelas, and lymphogranuloma venereum), Crohn disease, primary dysplastic angiopathy, bilateral varicose veins in the lower extremities, dermopathy from
Figure 2. Appearance of bilateral major labiectomy. A, Marked excision boundaries of lesion. B, Excised labia majora. C, Closed labiectomy field.
Copyright © 2014 American Society for Colposcopy and Cervical Pathology. Unauthorized reproduction of this article is prohibited.
Primary Vulvar Lymphangioma Circumscriptum
&
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Figure 3. Thin-walled, ectatic lymphatic channels in the papillary dermis. There is mild perivascular lymphocytic inflammatory infiltrate (hematoxylin-eosin stain; A, 25; B, 100; C, 200; D, 400).
penicillamine or corticosteroids, surgical trauma, keloids, scleroderma, and rhabdomyosarcoma [2, 19, 27]. Radiotherapy is generally being accused of being a causative factor for the secondary form of vulvar LC when there is a previous history of radiation treatment because of pelvic malignancies [15]. Because none of the etiologic factors mentioned above are shown in the present case, primary vulvar LC was diagnosed (see Figure 1). To date, in the English-language medical literature, primary LC of the vulva has been reported only in 21 patients, excluding the one reported in this article.
Figure 4. Appearance of vulva after 20 weeks of operation.
Clinically, LC is characterized by translucent vesicles, which may be scattered or grouped like frog-spawn. Rarely, the lesions can have a firm hyperkeratotic appearance. Vulvar LC can be asymptomatic, pruritic, burning, or painful. It may cause distressing symptoms of itching, pain, oozing of serous fluid, tendency to infections, psychosexual dysfunction, and cosmetic disfigurement [14, 15, 28]. Complications associated with vulval LC include cellulitis and psychosexual dysfunction. Follow-up of patients with lymphedema is recommended because transformation to lymphangiosarcoma may occur in the edematous limbs. Two cases of squamous cell carcinoma and lymphangiosarcoma arising within the LC have been reported [2]. Differential diagnosis of vulvar LC includes herpes zoster, molluscum contagiosum, genital warts, tuberculosis verrucosacutis (lupus verrucosus), leiomyoma, cellular angiofibroma, angiomyofibroblastoma, and aggressive angiomyxoma. If associated with significant hyperkeratosis, the clinical presentation may mimic condyloma acuminata. Biopsy specimen of the lesion forms the mainstay for diagnosis [17, 20, 24, 26]. There is no standard treatment of choice described for the management of vulvar LC. Depending on the type and extent of LC, the treatment options include procedures such as observation, abrasive modalities (carbon dioxide laser, liquid nitrogen, electrocoagulation, or sclerosing therapy), superficial radiotherapy, and surgical
Copyright © 2014 American Society for Colposcopy and Cervical Pathology. Unauthorized reproduction of this article is prohibited.
e4
&
KOKCU ET AL.
Table 1. Cases of Primary Vulvar Lymphangioma Circumscriptum Reported Until 2013 and the Applied Treatments No.
Date
Authors
Age, y
1 2
1981 1989
14 42
Radical vulvectomy Partial vulvectomy
3 4 5 6 7
1991
Bauer et al. [5] Abu-Hamad et al. [6] Johnson et al. [7]
32 35 20 76 20
Local Excision Vulvectomy Vulvectomy Patient refused treatment Local Excision (2 times)
8
1998
28
9
1999
24
Patient refused the treatment Observation
10 11 12 13 14 15
2001 2002 2006 2006 2007 2008
48 30 35 8 37 17
Simple vulvectomy Radical wide local excision Radical vulvectomy Sclerosing therapy Radiotherapy Major labiectomy
16 17 18
2008 2009
18 72 3
Major labiectomy Vulvectomy Observation
36 44 24
Vulvectomy Conservative treatment Physiotherapy
35
Major labiectomy
19 20 21 22
1992 1995 1995
Murugan [8] Cecchi et al. [9] Short and Peacock [10] Nishi [11] Gordon and Kaufman [12] Sah et al. [13] Vlastos et al. [14] Roy et al. [4] Ahn et al. [15] Yildiz et al. [16] Ghaemmaghami et al. [17]
Dikmen et al. [18] Aggarwal et al. [19] 2010 Basak et al. [20] 2012 Shah et al. [21] 2013 Chattranukulchai et al. [22] Present case Kokcu et al.
Treatment
excision (local excision, simple or extended vulvectomy) [2, 4, 16, 20]. Treating superficial lesions with methods such as carbon dioxide laser therapy and superficial radiotherapy has resulted in a short-term cure. Abrasive modalities have advantages of outpatient procedure, regional anesthesia, and repetition in case of recurrence [26]. These treatment modalities were applied to but rather secondary cases (only 2 of the primary cases). External radiotherapy was administered in only 1 case of primary vulvar LC and led to a surprisingly good response [16]. However, radiotherapy may cause premature ovarian failure and, very rarely, secondary malignancies. The disadvantage of abrasive modalities is a high recurrence rate (55.6% within an average period of 4.5 mo). Other complaints are oozing, lymphorrhea, and recurrent erysipelas. Hence, a close follow-up and early treatment of recurrence are a must for this modality [4]. The most preferred treatment for both the primary and secondary types is surgical excision. In surgical excision, the deep-feeding lymphatic cisterns should be removed to prevent recurrence [17]. Surgical management of vulvar lymphangioma offers a more permanent solution. However, even with the best treatment option,
recurrence is common. One should take adequate precautions to excise lymphatic cisterns in deep subcutaneous tissue for better results [26]. Fourteen of 22 cases of primary vulvar LC, including the one reported in this article, were treated surgically. Excisional surgical procedures for the treatment of primary vulvar LC varied from local excision to radical vulvectomy (see Table 1). In the present case, after bilateral major labiectomy, vulvar pain of the patient has disappeared as well, and the appearance of the vulva after 20 weeks of operation was normal (see Figures 2 and 3). The extent of the lesion also affects the surgical outcome and recurrence rates. The recurrence rate after the surgical procedure was 23.1% on variable follow-up ranging from 6 to 81 months [26]. The aim of this case report was to highlight an unusual and extremely rare case of vulvar disease. Primary vulvar LC poses a diagnostic challenge to treating gynecologists because of various clinical mimics. When diagnosed, even with the best treatment options, recurrences are common. Depending on the age of the patient and the extent of the lesion, local excision, major labiectomy, and vulvectomy are the preferred modes of treatment for primary forms especially.
REFERENCES 1. Shetty V, Venkatesh S. Acquired lymphangioma circumscriptum of the vulva. Int J Gynaecol Obstet 2012;117:190. 2. Amouri M, Masmoudi A, Boudaya S, Amouri A, Ben Ali I, Bouassida S, et al. Acquired lymphangioma circumscriptum of the vulva. Dermatol Online J 2007;13:10. 3. Esquivias Go´mez JI, Miranda-Romero A, Cuadrado Valle´s C, Bajo del Pozo C, Sa´nchez Sambucety P, Martı´nez Ferna´ndez M, et al. Lymphangioma circumscriptum of the vulva. Cutis 2001;67:229Y32. 4. Roy KK, Agarwal R, Agarwal S, Kumar S, Malhotra N, Gopendru N. Recurrent vulval congenital lymphangioma circumscriptumVa case report and literature review. Int J Gynecol Cancer 2006;16:930Y4. 5. Bauer BS, Kernahan DA, Hugo NE. Lymphangioma circumscriptumVa clinicopathological review. Ann Plast Surg 1981;7:318Y26. 6. Abu-Hamad A, Provencher D, Ganjei P, Penalver M. Lymphangioma circumscriptum of the vulva: case report and review of the literature. Obstet Gynecol 1989;73:496Y9. Review. 7. Johnson TL, Kennedy AW, Segal GH. Lymphangioma circumscriptum of the vulva. A report of two cases. J Reprod Med 1991;36:808Y12. 8. Murugan S. Lymphangioma circumscriptum of the vulva. J Indian Med Assoc 1993;91:77.
Copyright © 2014 American Society for Colposcopy and Cervical Pathology. Unauthorized reproduction of this article is prohibited.
Primary Vulvar Lymphangioma Circumscriptum
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