An Account of a Giant Adolescent Vulvar Lipoma

Case Report

Lipomas though the most common benign soft tissue tumors of mesenchymal origin, are rarely found in the vulva.¹²³⁴ They are commonest in the fourth to sixth decade of life² and more often noted in the right labia majora.³ Owing to the rarity of their occurrence and delay in seeking medical care due to associated social stigma in this part of the world, they often pose a diagnostic and therapeutic dilemma to the surgeon. We here discuss the case of an adolescent female who presented with an enormous (26.5 × 21.5 × 6.5 cm) vulvar lipoma and had a satisfying clinical and esthetic surgical outcome.

A 15-year-old girl presented to the pediatric surgical outpatient department after claiming to have recently noticed a left-sided vulvar mass that had grown in size over the last 1 year. She complained of a dragging type of pain and discomfort while walking due to the giant hanging mass. She was not sexually active, and she had normal menstrual cycles. On careful enquiry, her mother disclosed that her left labia majora was larger than the right since birth. The patient later revealed that the reason she did not seek medical attention earlier was that she was embarrassed to discuss the issue with her family.

On inspection, a soft to firm, nontender, pedunculated swelling of size 26 × 20 cm involving the left inguinolabial region was noted with stretched skin and sparse hair distribution over it (Fig. 1). There was a cough impulse present over the inguinal component; however, it was in continuity with the rest of the swelling and was not reducible. The posteroinferior aspect had some skin discoloration and thickening. The vulvar anatomy was markedly distorted owing to the size, the labia minora could not be separately demarcated, and the clitoris was completely hidden.

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Fig. 1 Clinical photographs showing the swelling.

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A magnetic resonance imaging (MRI) was done to characterize the lesion, delineate the anatomy, and rule out differentials including a soft tissue sarcoma and an omentocele. The MRI showed a large predominant fat signal intensity lesion which confirmed the clinical suspicion of a lipoma (Fig. 2). The patient was advised to allow growth of her pubic hair to guide surgical reconstruction of the labia minora and majora. Six weeks later, she underwent surgical excision (Fig. 3) with labial reconstruction (Fig. 4). The histopathology report was consistent with that of a lipoma. The perioperative period was uneventful, and she is doing well at 1 year of follow-up with significant improvement in her academic performance and self-esteem.

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Fig. 2 T2 (fat enhancing) and T2 turbo inversion recovery magnitude (fat suppressed) coronal views of the lesion on MRI.

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Fig. 3 Postoperative images of the vulva immediately and 1 month after the surgery.

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Fig. 4 Excised lipomatous lesion.

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Discussion

Though more common in the fourth to sixth decade,²⁵ vulvar lipomas can occur in any age group ranging from newborns³ to the elderly.⁶ The etiology however depends on the age of presentation. Those seen in the newborn period and pediatric population are congenital, whereas those presenting in adulthood are usually acquired due to tissue trauma from chronic irritation.²⁵ Our patient had a combination of these factors, the increase in size probably being due to repeated minor trauma from the size being so large.

Vulvar lipomas are known to grow to enormous sizes with pedunculation owing to the combined effect of gravity and delay in seeking medical attention. Patients hailing from socially conservative backgrounds often have inhibitions in seeking medical care for problems concerning the genitalia. Many a times they report to the hospital only when they face significant interference with activities of daily living. The previously largest reported vulvar lipomas in literature had a maximum diameter of 20⁷ and 23 cm,⁸ respectively. This patient's lipoma was 26.5 cm in its greatest dimension which makes it the largest documented vulvar lipoma among both adults and children. Only nine case reports have been published regarding presentation of a vulvar lipoma in the pediatric age group till date, all with sizes much smaller than that in the present case (Table 1).

Currently, there is no literature to suggest why the right labia is more common than the left. We postulate that this has to do with canal of Nuck abnormalities being more common on the right side.¹⁴¹⁵ Further studies however will be required to confirm whether there is actually a relation between the two.

The role of imaging lies in ruling out other differentials while delineating the extent and revealing a possible malignancy (in which case a fine needle aspiration/biopsy would be needed to confirm the diagnosis). The common differentials to be considered include Bartholin's cysts, cysts of the canal of Nuck, inguinal hernias, and rarely liposarcomas.²³⁴ Computed tomography scans and MRI have been used with greater frequency in the recent past for better characterization of the lesions and identifying the proximity of surrounding structures.¹²³⁵⁷ Ultimately, histopathology confirms the diagnosis and rules out malignancy. When large, a detailed multisectional examination is crucial.

Universally, surgical excision is considered to be the ideal modality of treatment¹³; however, reconstruction of the vulva needs to be given consideration, especially in the pediatric and adolescent age group where there is a significant psychological impact on the patient. Utilizing the pubic hair growth pattern post puberty comes in handy when planning the surgical incision. We found that allowing growth of pubic hair before surgery helped delineate the boundaries of the labia majora and minora. Hair provides the additional advantage of masking the scar and enhancing cosmesis. The patient is doing clinically well at 1 year of follow-up with significant improvement in her confidence and self-esteem.

Conclusion

The vulva is a rare location for lipomas more so in the pediatric population. When social stigma is a concern, there is delay in seeking medical care leading to progression of growth thereby making management challenging. Even with a delayed presentation, a complete surgical excision with a cosmetically acceptable outcome can be achieved.