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Journal of Indian Association of Pediatric Surgeons
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Table of Contents   
CASE REPORT
Year : 2017  |  Volume : 22  |  Issue : 3  |  Page : 163-164
 

Clitoral choristoma: A rare case report


1 Department of Paediatric Surgery, IGICH, Bengaluru, Karnataka, India
2 Department of Pathology, IGICH, Bengaluru, Karnataka, India

Date of Web Publication8-Jun-2017

Correspondence Address:
Jayalaxmi Shripati Aihole
Assistant Professor Indira Gandhi Institute of Child Health, Bangalore, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-9261.207642

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   Abstract 

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.


Keywords: Choristoma, clitoral cyst, clitoromegaly


How to cite this article:
Aihole JS, Lokanath H, Babu M N, Deepak J. Clitoral choristoma: A rare case report. J Indian Assoc Pediatr Surg 2017;22:163-4

How to cite this URL:
Aihole JS, Lokanath H, Babu M N, Deepak J. Clitoral choristoma: A rare case report. J Indian Assoc Pediatr Surg [serial online] 2017 [cited 2019 Sep 21];22:163-4. Available from: http://www.jiaps.com/text.asp?2017/22/3/163/207642



   Introduction Top


Choristoma, a congenital anomaly, is better described as a “heterotopic” rest of cells.[1],[2] 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 Top


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 1]a.
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

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Otherwise, the external genitalia were unremarkable with normal vaginal and anal orifices, with no signs of virilization. There was no hyperpigmentation [Figure 1]a and [Figure 1]b. Magnetic resonance imaging revealed swelling with fat intensity signals continuous with clitoral fibrous core suggestive of clitoral hamartoma [Figure 1]d and [Figure 1]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 1]c. 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 2]a and [Figure 2]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

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   Discussion Top


The term choristoma was first introduced by Krolls et al. in 1971.[1],[2] A choristoma is a benign, congenital proliferation of histologically mature tissue elements not normally present at the site of occurrence.[1],[2],[3],[4] 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.[3],[4]

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.[3],[4] 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.[3],[4],[5]

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.[2],[5]

The clitoris can be involved in a variety of benign and malignant neoplasms.[6],[7],[8] 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.[6],[7],[8]

In our case, the soft, mobile, nontender swelling was arising from only clitoral hood without involving corporal bodies [Figure 1]a and [Figure 1]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 2]a and [Figure 2]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.

Acknowledgment

We would like to thank all pediatric surgical colleagues, anesthetists, OT Staffs, and dermatologist, IGICH, Bangalore, for their kind support and encouragement.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Krolls SO, Jacoway JR, Alexander WN. Osseous choristomas (osteomas) of intraoral soft tissues. Oral Surg Oral Med Oral Pathol 1971;32:588-95.  Back to cited text no. 1
    
2.
Suganya R, Malathi N, Vijaya Nirmala S, Ravindran C, Thamizhchelvan H. Cartilaginous choristoma of the gingiva: A rare clinical entity. Case Rep Dent 2014;2014:246965.  Back to cited text no. 2
    
3.
Strayer DS, Rubin E. Neoplasia. In: Rubin R, Strayer DS, editors. Rubin's Pathology: Clinicopathologic Foundations of Medicin. 6th ed. Philadelphia: Wolter Kluwer and Lippincott, Williams and Wilkins; 2012. p. 157-212.  Back to cited text no. 3
    
4.
Kumar V, Abbas AK, Fausto N, Aster J. Neoplasia. In: Robbins and Cotran Pathologic Basis of Disease. 8th ed. Philadeplhia: Elsevier Saunders; 2010. p. 174-6.  Back to cited text no. 4
    
5.
Lavien G, Suson KD, Kim B, Wang MH. Presentation of a clitoral mass in a prepubescent female: A case report and discussion of the evaluation. Clin Med Insights Pediatr 2015;9:65-6.  Back to cited text no. 5
    
6.
Schmidt A, Lang U, Kiess W. Epidermal cyst of the clitoris: A rare cause of clitorimegaly. Eur J Obstet Gynecol Reprod Biol 1999;87:163-5.  Back to cited text no. 6
    
7.
Abudaia J, Habib Z, Ahmed S. Dermoid cyst: A rare cause of clitorimegaly. Pediatr Surg Int 1999;15:521-2.  Back to cited text no. 7
    
8.
Teague JL, Anglo L. Clitoral cyst: An unusual cause of clitorimegaly. J Urol 1996;156:2057.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2]



 

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    References
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