Archives of International Surgery

CASE REPORT
Year
: 2013  |  Volume : 3  |  Issue : 2  |  Page : 158--160

Cystic lymphangioma of the vulva: A rare case report


Anandhi Amaranathan1, Sankappa Sinhasan2, Simon D Dasiah1, Basavanandswami Hartimath1,  
1 Department of Surgery, Indira Gandhi Medical College and Research Institute, Pondicherry, India
2 Department of Pathology, Indira Gandhi Medical College and Research Institute, Pondicherry, India

Correspondence Address:
Anandhi Amaranathan
Department of Surgery, Indira Gandhi Medical College and Research Institute, Pondicherry - 605 009
India

Abstract

Lymphangioma is a benign congenital malformation, most commonly diagnosed in infants and children. The vulva is a rare site for the development of lymphangioma. Around 30 cases of lymphangioma circumscriptum (capillary variety) have been reported, only 7 cases of cystic lymphangioma have been reported in the literature so far. We report a case of vulval, multiloculated, cystic lymphangiomas in a 27 year old female, for its rarity and to consider this in the differential diagnosis of a vulval pathology. Viewing vulval lymphangiomas as a differential diagnosis in mind while examining a vulval swelling, will help in proper plan of complete excision.



How to cite this article:
Amaranathan A, Sinhasan S, Dasiah SD, Hartimath B. Cystic lymphangioma of the vulva: A rare case report.Arch Int Surg 2013;3:158-160


How to cite this URL:
Amaranathan A, Sinhasan S, Dasiah SD, Hartimath B. Cystic lymphangioma of the vulva: A rare case report. Arch Int Surg [serial online] 2013 [cited 2019 Oct 17 ];3:158-160
Available from: http://www.archintsurg.org/text.asp?2013/3/2/158/122948


Full Text

 Introduction



Lymphangioma is a benign congenital malformation, most commonly diagnosed in infants and children less than 2 years of age. [1] Its occurrence in adults is extremely rare. And so far approximately 100 cases have been reported in the adults, generally in the head and neck region (75%), axilla (20%), mediastinum (5%), groin, retroperitoneal space, and pelvis. [2] Lymphangiomas are considered to be the result of sequestration of lymphatic tissue that has retained its potential for growth. The three variants which have been described are: Capillary-characterized by small thin walled vascular channels; Cavernous-large channels with a fibrous coat; and Cystic-large cystic endothelial lined spaces. [2]

The vulva is a rare site for the development of lymphangioma. Around 30 cases of lymphangioma circumscriptum (capillary variety) have been reported. But only 7 cases of cystic lymphangioma have been reported in the literature so far up to March 2013. We present a case of cystic lymphangioma which is a very rare entity in the adult population. We report this case for its rarity and its clinical implications in the diagnosis of a vulva swelling.

 Case Report



A 27-year-old female presented to the surgical outpatient clinic with complaints of swelling in the external genitalia of 6 months duration. Swelling was spontaneous in onset, slowly increased in size and was associated with occasional pain and discomfort. There was no history of trauma, fever or radiotherapy. However, there was a history of surgery in the right inguinal crease for skin lesion at her younger age, for which the details were not available. The scar mark of previous surgery was seen over right inguinal area [Figure 1].{Figure 1}

On examination there was a 7 × 5 cm swelling noted in the right labia majora. The consistency was variable ranging from firm to cystic. The swelling had ill-defined margins and showed restricted mobility. Skin over the swelling was normal except a scar mark of previous surgery seen just below the lower margin of the swelling in the right inguinal crease. No other relevant clinical features found. Provisional diagnosis of lipoma was made. However, differential diagnosis of fibroadenoma arising from ectopic breast tissue (along milk line) was also considered. Ultra-sonogram showed cystic lesion with multiple septae. Fine needle aspiration cytology (FNAC) was carried out, where 5 ml of straw coloured clear fluid was aspirated. Smears studied from the sediment of aspirated fluid showed predominantly lymphocytes with occasional cyst macrophages, against a proteinaceous background suggestive of lymphangioma [Figure 2]. Complete excision of the lesion was done. Intra-operatively, a multilobulated subcutaneous mass was noted with illdefined capsule, surrounded by dense adhesions.{Figure 2}

Grossly, the specimen measured 7 × 5 × 3 cm, irregular in shape and spongy to fibrous in consistency. Cut section showed multiloculated cystic mass [Figure 3] with cysts ranging from 1 mm to 2 cm in size and filled with straw colour fluid. The lesion was predominantly fibrous with foci of adipose tissue scattered in between. Areas of hemorrhages were also seen. Microscopy revealed a hamartomatous lesion composed of capillary and cavernous sized vascular channels [Figure 4] lined by endothelial cells with lumen of cystic spaces containing hemorrhage as well as lymph. Focal aggregates of lymphocytes were seen infiltrating the fibrocollagenous stroma. The stroma also showed irregularly arranged smooth muscle bundles and many capillary sized vascular spaces. A diagnosis of Hamartoma-Cystic Lymphangioma of Vulva was made. Patient's post-operative period was uneventful and 1 year followup showed no recurrence of the lesion. {Figure 3}{Figure 4}

 Discussion



Lymphangioma is a benign congenital malformation, produced by sequestered primitive lymphatic tissue that fails to communicate with peripheral drainage pathways. The congenital form of lymphangioma is the commonest form, diagnosed most commonly under the age of 2 years. [1] There are various theories regarding the development of lymphangioma and its classification. It usually occurs in neck (75%), axilla (20%), mediastinum (5%), groin, retroperitoneal space, and pelvis. [2] Even though it is rare in adults, around 100 cases of lymphangiomas are reported, which may be an acquired form, secondary to radiation, surgery, trauma, keloid, scrofuloderma, pregnancy, scleroderma, neoplasia, or infections such as filariasis, tuberculosis, recurrent erysipelas and lymphogranuloma venereum, in which case it is termed as lymphangiectasias. [3]

The most widely accepted theory about the development of cystic lymphangioma is that they arise from sequestrations of the primitive embryonic lymph sacs. However, the aetiology in the adult population is controversial. Some authors attribute adult lymphangioma to delayed proliferation of the congenital or acquired lymphoid rests following trauma or preceding respiratory infection. [4] Others dispute the congenital origin and propose that adult cystic lymphangiomas arise as a result of trauma alone. [5] Our patient had surgery in right inguinal region, (the nature of the lesion not known) which could have caused the traumatic insult.

Lymphangiomas are classified microscopically into three categories and some authors have added benign lymphangioendothelioma as the fourth category: 1) Lymphangioma simplex (capillary lymphangioma) (lymphangioma circumscriptum) composed of small, thin-walled lymphatics; 2) cavernous lymphangioma comprising dilated lymphatic vessels with surrounding adventitia; 3) cystic lymphangioma (cystic hygroma) consisting of huge, macroscopic lymphatic spaces with surrounding fibrovascular tissues and smooth muscle; and 4) benign lymphangioendothelioma (acquired progressive lymphangioma), in which lymphatic channel dissects through dense collagen bundles. Occasionally, channels may be filled with blood, a mixed hemangiolymphangioma, an uncommon developmental anomaly with a propensity to invade underlying tissues and to recur locally, distinguishing it from the simple lymphangioma or hemangioma. [6] In general, mucosal lesions belong to the first type (capillary lymphangioma) and are hard to eradicate with surgery, whereas cystic lymphangioma, the most common type, also known as cystic hygromas, are macrocystic and easier to resect. The latter type of malformation presents as painless non-pulsatile masses with a rubbery consistency that are covered by normal colour skin. [7]

Diagnosis is best made by clinical examination. Imaging is of little help in such superficial well-defined lesions. Fine needle aspiration cytology is of some help in formulating a preliminary diagnosis. The correct diagnosis is possible only after the histopathological examination of the surgically resected specimen. [8] Surgical excision of lymphangioma is the best way of treatment, and there is an increased possibility of recurrence if excision is not performed completely. [9]

 Conclusion



Cystic lymphangioma of the vulva in adult population is a rare occurrence. In spite of a great improvement in the investigating modalities, the correct pre-operative diagnosis may pose a diagnostic challenge. Therefore, this diagnosis should be considered when examining a vulval swelling so that we can plan for complete excision to prevent recurrence.

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