Aijaz, Farrukh

Hassan, Sheema H.

Pervez, Shahid

Wasti, Shehnaz

eCommons@AKU

eCommons@AKUDepartment of Pathology and Laboratory Medicine Medical College, PakistanJune 1993Aggressive Angiomyxoma of VulvaFarrukh AijazAga Khan UniversityShahid PervezAga Khan University, shahid.pervez@aku.eduShehnaz WastiAga Khan UniversitySheema H. HassanAga Khan UniversityFollow this and additional works at: http://ecommons.aku.edu/pakistan_fhs_mc_pathol_microbiolPart of the Female Urogenital Diseases and Pregnancy Complications Commons, Obstetrics andGynecology Commons, Oncology Commons, Pathology Commons, and the Women's HealthCommonsRecommended CitationAijaz, F., Pervez, S., Wasti, S., Hassan, S. H. (1993). Aggressive Angiomyxoma of Vulva. Journal of Pakistan Medical Association, 34(6),123-125.Available at: http://ecommons.aku.edu/pakistan_fhs_mc_pathol_microbiol/314Aggressive Angiomyxoma of VulvaPages with reference to book, From 123 To 125 Farrukh Aijaz, Shahid Pervez, Shehnaz Wasti, Sheema H. Hassan ( Departments of Pathology and Gynaecology, The AgaKhan University Hospital, Karachi. ) Aggressive angiomyxoma is an infrequent tumour of the vulva of which only single ease and smallseries have been reported in the literature1-4. The biological behaviour of this lesion is variable. Inmany patients, the tumour is benign, although an aggressive outcome with recurrences is common.Metastasis has not been described. We report three cases of aggressive angiomyxoma of the vulva thatclinically presented as large bulky polypoidal masses.Clinical and pathological Findings Case 1A 16-year old unmarried female presented with a history of vulval mass in the labial region which hassteadily grown since last two years. Over a period of 5 months prior to her visit, she had fever off andon and was seen by a number of doctors and was prescribed a wide array of drugs including variousantibiotics, antitubercubus drugs and steroids. She also had an echocardiogram elsewhere which wasinterpreted as chronic pericarditis with mild effusion suggestive of tuberculosis. Apparently, her vulvalmass was never examined. At the time of presentation, the mass showed extensive skin ulceration withpurulent discharge arising from right labia major a (Figure 1).Pelvic examination revealed a normal sized uterus and no adnexal mass was felt. Fler haemoglobin was7.6 g/dl and she was transfused four units of packed cells before the surgery for the excision of themass. No recurrence was reported till the last follow-up one year after surgery. The surgical specimenweighed 860 gin and measured 2 1x15x1 1 cm. The cut surface was gelatinous white and soft.Microscopically, the tumour consisted of myxoid tissue with enmeshed benign, uniform stellate cellswhich lacked atypia. The myxoid stroma was moderate to markedly vascular consisting of bloodvessels of variable caliber (Figure 2).The majority of vessels had dilated lumina with small foci of fresh hemorrhages in the myxoid stroma.Acute and chronic inflammation was seen only in areas immediately adjacent to skin ulceration. Nomitosis or nuclear atypia was noted in stellate stromal cells. Immuno histochemical studies revealedthese stromal cells positive for vimentin but negative for actin and desmin.Case 2A 36-year old married female who presented with a history of progressively increasing swelling in theleft labial region for the last four years. She experienced discomfort on sitting and mild dyspareunia.Examination revealed a soft mass arising from the left labia majora. There was no discharge, oozing orulceration. Bimanual examination and ultrasound revealed a normal pelvis. The tumour was excisedunder general anaesthesia. Postoperative recovery was uneventful. No recurrence was noted for a year,but then she was lost to follow-up. The surgical specimen measured 13x10x8. 5 cm. The overlying skinwas intact and unremarkable. Cut surface was light grey white, gelatinous with small focalhaemorrhages. Flistological features were almost identical to that of case 1, however, the vascular wallstended to be thicker and there were foci of perivascular lymphocytic aggregates. Focal areas offibrosiswere also present. On immune histochemistry, the stromal cells were positive for vimentin but werenegative for actin and desmin.Case 3A 35-year old married female gave a 10-year history of a slowly growing mass in right labia majora.The mass was excised under general anaesthesia. The post-operative period was uneventful. The massmeasured 10.5x5.5x4.5 cm. No overlying skin was present in the specimen. The cut surface was softand gelatinous with many mucoid and cystic areas. Histological findings were similar to the above twocases. In addition, however, the vascularity tended to be angiomatous. Small foci of capillaryhemangioma like areas were separated by vascular myxoid areas (Figure 3).No atypia or mitosis were noted in the stellate stromal cells.Discussion Aggressive angiomyxoma was the term coined by Steeper and Rosai for a distinctive type ofgynaecologic myxoid and vascular neoplasm1. The term ‘aggressive’ denoted its propensity to recur ifnot adequately excised. Reliable estimates of recurrence rates are not known. To date, no metastaticlesion has been documented. Lesions with perhaps similar pathology had been identified before.Virchow described a labial lesion as ‘myxoma’ which later on recurred2. Various lesions were thoughtas ‘fibromas’ and ‘liposarcomas’ with myxoid change attributed to degeneration3. Aggressiveangiomyxoma diagnosed as such, is an exceedingly rare neoplasm and this is perhaps largely due tonon-recognition. Our three cases measured more than 10 cm in greatest dimension. The size range inSteeper’s1 series was 3-60 cm. One of our patients (case 1) was only 16 years old while in Steeper’sseries, the age range was 21-38 years. Aggressive angiomyxoma has also been reported in pelvic softparts4, retroperitoneum4, umbilical cord5, levator hernia6 and scrotum7. However, its predominantlocation is in vulva and perineum and this is probably due to relatively myxoid and vascular stromanormally present at these sites. The neoplasm lacks encapsulation and may infiltrate the neighbouringareas causing inadequate excision and tendency to recur. The neoplasm has two basic histologicfeatures, the myxoid stroma with benign stromal cells lacking mitosis and nuclear atypia. The morecharacteristic feature is its vascularity. The vessels are part of the tumour, moderately abundant, usuallypresent as dilated capillaries, but lacks the anastomosing pattern. Thickening of the vascular smoothmuscles can also occur. In one of our cases (case 3), capillary hemangioma like areas were widelyseparated by vascular myxoid stroma. The stromal cells were thought of fibroblastic or myofibroblasticon the basis of immune histochemical and ultrastructural investigations8. Our immunohistochemicalfindings are in accord with other investigators. Entrapped glandular elements9 were not seen in ourcases. The differential diagnosis of aggressive angiomyxoma includes other myxoid neoplasms such asmyxomas, myxoid malignant fibrous histiocytoma, embryonal rhabdomyosarcoma (sarcomabotyvoides), myxoid liposarcomas, nerve sheath myxomas and leiomyomas with myxoid change,especially during pregnancy. However, in most cases, the diagnosis should not be difficult as long asone is aware of the existence of this entity and carefully excludes other diagnostic possibilities.Immunoperoxidase studies may be helpful in difficult cases. Smaller tumours are apt to be confusedwith benign vulval fibroepithelial polyps which paradoxically tend to have atypical stromal cells andwill lack the peculiar vascularity of aggressive angiomyxoma.Acknowledgement We are grateful to Mrs. Amina Anwerali for her secretarial and Ms Masooma Naqvi for her technicalhelp.References 1. Steeper, T., Rosai, J. Aggressive angiomyxoma of female pelvis and perineum. Am.J. Surg. PathoL1983;7:463-75.2. Virchow, R. Cellular pathology aa based upon phyaiologieal and pathological histology.Philadelphia, J.B. Lippincott, 1983, pp. 525-26.3. Fisher, W.H. Myxoma. Ann. Surg., 1919;69:596-99.4. Begin, L.R., Clement, P.B., Kirk, ME., Jothy, S., MeCaughey, W.T.E., Ferenezy, A. Aggressiveangiomyxoma of pelvic aoft parts: a clinieopathologieal atudy of nine cases. Hum. Pathol.,1985:16:621-28.5. Jauniaux, B., Mosr.oso, G., Chitty, L, Gibb, D., Driver, M., Campbell, S. An angiomyxoma involvingthe whole length of the umbilical cord: prenatal diagnosis by ultrasonography. 3. Ultrasound. Med.,1990;9:419-22.6. Sutton, G.P., Rogers, RE., Roth, L.M., Ehrlich, CE Aggressive angiomyxoms lirst diagnosis aslevator hernia. Ami. Obstet. Gynaecol., 1989;161:73-75.7. Moreno Aviles, J., Salinas Sanchez, S., Alcala-Santanella, Casanova, C., Guardiola, Mae, A., Server,Falgas, 0. Anglomyxoma invaslo de escroto. Arch. Esp. UroL, 1990;43:908.8. Mandai, K., Moriwaki, S., Motoi, M.Aggressiveangiomyxoma of thevulva: reportofa case. Acta.Pathol. Jpn., 1990;40:927-34.9. Semanta, AR., Crambini, C., Borgiani, L., Comes, P. Aggressive angiomyxoma of the pelvis andperineum: reportofa casewith immunohistological and electron microscopic study. Pathologies,1989;81(1074):463-69.

Aggressive Angiomyxoma of Vulva

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Aggressive Angiomyxoma of Vulva

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