Clitoral Choristoma: A Rare Case Report.

Clitoral hypertrophy is usually seen as a congenital malformation, specifically during the stages of hormonal expressions in the disorders of sexual development. Acquired clitoral hypertrophy is a relatively rare condition. Clitoris can be the site of neoplastic lesions. Clitoral choristoma has not been reported so far in the literature.

INTRODUCTION

Choristoma, a congenital anomaly, is better described as a “heterotopic” rest of cells.[12] Choristoma is a more acknowledged term and it was first described by Krolls et al. as tumor-like growths of microscopically normal tissue in an unusual location.[1] Clitoral choristoma as per our knowledge has not been described in the literature so far.

CASE REPORT

A 6-year 4-month-old female child was brought to us with history of introital mass noticed since birth by her mother. The swelling was gradually increasing in size, not associated with pain or any discharge. The child did not have any bowel and bladder disturbances. On examination, a soft to firm swelling of size 2 cm × 1 cm was noted at the clitoral skin hood, just above the clitoral glans and the urethral orifice [Figure 1a].

Figure 1

(a) Clinical appearance of clitoral choristoma covering the half of the vestibule. (b) Clitoral hood choristoma. (c) Immediate postoperative appearance with an infant feeding in the urethra. (d) T2 axial image at the level of clitoris showing clitoral choristoma. (e) Fat saturation sequence showing corresponding axial image of the clitoris: showing clitoromegaly with yellow arrow indicating fat contents

Otherwise, the external genitalia were unremarkable with normal vaginal and anal orifices, with no signs of virilization. There was no hyperpigmentation [Figure 1a and b]. Magnetic resonance imaging revealed swelling with fat intensity signals continuous with clitoral fibrous core suggestive of clitoral hamartoma [Figure 1d and e]. Endocrinological evaluation was normal. Surgical procedure included reverse “V” incision over the dorsal surface of the swelling and dissection from subcutaneous tissues was done. The cyst was excised without any damage to the deep fascia and neurovascular bundles. Excessive skin was trimmed [Figure 1c]. The child had an uneventful postoperative recovery. The histopathology revealed predominately adipose tissue having multifocal dispersion of mature skeletal muscle bundles with neural bundles, vessels, and fibrous tissue [Figure 2a and b], suggestive of choristoma.

Figure 2

Histopathological appearances – (a) yellow arrow indicating mature skeletal muscle bundles, blue arrow indicating mature adipose tissue. (b) Long green arrow indicating the fibrous tissue. Yellow, triangular pointer indicating mature nerve bundle. White triangular pointer indicating the vessel

DISCUSSION

The term choristoma was first introduced by Krolls et al. in 1971.[12] A choristoma is a benign, congenital proliferation of histologically mature tissue elements not normally present at the site of occurrence.[1234] This heterotopic congenital lesion results from normal tissue migrating to or remaining in an abnormal location during embryogenesis (hence, the derivation from the Greek word for “separated mass”). Like hamartomas, they gain a certain size and then cease to grow.[34]

Choristomas are not tumors although their gross or microscopic appearance may resemble that of tumors. Choristomas should also be distinguished from hamartomas which are tumor-like malformations composed of a focal overgrowth of mature normal cells located where they are normally found.[34] They rarely invade or compress surrounding structures significantly. Because of their benign nature and excellent outcome, they need to be differentiated from other neoplasms.

Choristomas have been reported more commonly in head and neck region and gastrointestinal tract. Examples of choristomas in children include gastrointestinal duplication cysts with gastric rests, pancreatic rests in their walls, and adrenal tissue in the renal cortex.[345]

Disorders of sexual development are one of the most common causes for clitoromegaly. The other causes of androgen excess, which is the main contributing factor for clitoral enlargement, include functional endocrine tumors and exogenous hormonal intake.[25]

The clitoris can be involved in a variety of benign and malignant neoplasms.[678] Among the benign neoplasms, fibroma, leiomyoma, angiokeratoma, hemangioma, neurofibromatosis, keratoacanthoma and pseudolymphoma, hemangiopericytoma, granular cell tumors are reported. In adults, the most common malignancy is squamous cell carcinoma of the vulva invading the corporal bodies.[678]

In our case, the soft, mobile, nontender swelling was arising from only clitoral hood without involving corporal bodies [Figure 1a and b] and the absence of virilizing signs was excised completely with excellent outcome. Findings of mature adipose tissue with multifocal dispersion of mature skeletal muscle bundles which were not normally present in the clitoris confirmed the diagnosis of choristoma [Figure 2a and b].

However, clitoral choristoma has not been reported in the literature so far. They have an excellent outcome, following surgical excision.

Goals of the surgical management of clitoral choristoma include total excision of the lesion with emphasis on alleviation of patient's discomfort, clitoral sensation, and future comfortable sexual function.

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Conflicts of interest

There are no conflicts of interest.