A giant aggressive angiomyxoma of the vulva in a dwarf: a rare case report

Authors

  • Shubhra Mukherjee Department of Obstetrics and Gynecology, ESIC Model Hospital, Indore, Madhya Pradesh, India
  • Advait Prakash Department of Pediatric Surgery, SAIIMS, Indore, Madhya Pradesh, India
  • Hemlata Sodhiya Department of Obstetrics and Gynecology, Indore, Madhya Pradesh, India
  • Sarika Jaiswal Department of Obstetrics and Gynecology, Indore, Madhya Pradesh, India

DOI:

https://doi.org/10.18203/2320-1770.ijrcog20230549

Keywords:

Dwarf, Angiomyxoma, Thyroid agenesis

Abstract

Aggressive angiomyxoma is a rare soft tissue tumour that carries a high risk of local relapse. The tumour usually arises from the pelvic and perineal regions. We presented a case of a young girl with dwarfism, mental retardation and thyroid agenesis who presented with aggressive angiomyxoma of the vulva. As it is almost exclusively seen in women of reproductive age group, this is the first case reported in a teenage girl, to the best of our knowledge. A mentally retarded girl, aged fifteen years, presented with a large mass hanging from the vulva, with ulceration over the mass. She had short stature, anasarca, mental retardation, primary amenorrhoea, under-developed secondary sexual characters .and thyroid agenesis. Local examination revealed a pedunculated mass of 15 by 4 cm size arising from the mons pubis closely resembling a giant penis. Full hormonal and radiological work-up was done. Her condition was optimized and the mass was removed with wide excision of margins followed by cystoscopy under general anaesthesia. Histopathology and immunohistochemistry were suggestive of aggressive angiomyxoma. She has no recurrence till date. Despite its rarity, angiomyxoma should be considered in the differential diagnosis of any painless swelling located in the genitofemoral region. The principal treatment is surgical excision. Long term follow-up is needed due to its high tendency of local recurrence.

References

Nakamura T, Miura K, Maruo Y, Sunayama K, Maruyama K, Kashiwabara H, et al. Aggressive angiomyxoma of the perineum originating from the rectal wall. J Gastroenterol. 2002;37(4):303-8.

Salman MC, Kuzey GM, Dogan NU, Yuce K. Aggressive angiomyxoma of vulva recurring 8 years after initial diagnosis. Arch Gynecol Obstet. 2009;280(3):485-7.

Minagawa T, Matsushita K, Shimada R, Takayama H, Hiraga R, Uehara T, et al. Aggressive angiomyxoma mimicking inguinal hernia in a man. Int J Clin Oncol. 2009;14(4):365-8.

Steeper TA, Rosai J. Aggressive angiomyxoma of the female pelvis and perineum. Report of nine cases of a distinctive type of gynecologic soft-tissue neoplasm. Am J Surg Pathol. 1983;7(5):463-75.

Gaurav A, Gill P, Khoiwal K, Chowdhuri S, Kapoor D, Chaturvedi J. Aggressive angiomyxoma of the vulva- a rare entity: case report and review of literature. Int J Reprod Contracept Obstet Gynecol. 2020;9:2605.

Lourenço C, Oliveira N, Ramos F, Ferreira I, Oliveira M. Aggressive angiomyxoma of the vagina: a case report. Rev Bras Ginecol Obstet. 2013;35(12):575-82.

Joseph S, Helm J, Villegas E, Figueroa-Bodine J, Anderson J. Aggressive angiomyxoma: a rare cause of a vulvar mass. J Med Oncol Ther. 2020;5:81-3.

Das BP, Baruah D, Medhi KB, Talukder B. An aggressive angiomyxoma of vulva - A rare entity - A case report. J Midlife Health. 2016;7(3):140-3.

Blandamura S, Cruz J, Faure VL, Machado PI, Ninfo V. Aggressive angiomyxoma: a second case of metastasis with patient's death. Hum Pathol. 2003;34(10):1072-4.

Bégin LR, Clement PB, Kirk ME, Jothy S, McCaughey WT, Ferenczy A. Aggressive angiomyxoma of pelvic soft parts: a clinicopathologic study of nine cases. Hum Pathol. 1985;16(6):621-8.

Siassi RM, Papadopoulos T, Matzel KE. Metastasizing aggressive angiomyxoma. N Engl J Med. 1999;341(23):1772.

Downloads

Published

2023-02-27

Issue

Section

Case Reports