Ovarian myxoma

~IEDICAL INTELLIGENCE 40. Boecker, W. R., ttossfeld, D. K., Gallmeier,W. M., and Schmidt, C. G.: Clonal growth of Hodgkin's cells. Nature, 258:235-236, 1975. 41. Dorfman, R. F.: Enzyme histochemistryof normal, hyperplastic and neoplastic lymphoreticular tissues. In Roulet F. C. (Editor): Symposium on Lymphoreticular Tumors in Africa. Basal, S. Karger A. G., 1964, pp. 304-326. 42. Lukes, R.J., and Collins,R. D.: New approaches to the classification of the lymphomata. Br. J. Cancer, 31 (Suppl. 11):1-28, 1975. OVARIAN MYXOMA BtlAGIRATtI MAJMUDAR, M.D.,* PETER STEPHEN KAPERNICK, M.D.,? AND ROGER STEVE PHILHPS, M.D.~ Abstract A case of primary ovarian myxoma is reported. The tumor is rare as there are only two previously reported cases in the Ifferature. T h e term "myxoma" was first i n t r o d u c e d by Virchow t in 1863 and was m o r e precisely defined by him in 1871.-" H e noted that there a r e a n u m b e r o f tumors that r e p r o d u c e the structure o f the umbilical cord. H e believed that the t u m o r contained mucin in its intercellular substance and called the tissue o f the umbilical cord a n d its analogues nmcinous tissue. T u m o r s r e p r o d u c i n g this tissue were designated by him as mucinous tumors o r myxolnas. Although myxomas are known to arise from the soft tissue, 35 p r i m a r y ovarian myxoma is exceedingly rare, only two cases having previously been r e p o r t e d in the world literature.6, 7 CASE R E P O R T A 25 year old female, p a r a 4, was f o u n d to have an adnexal mass on routine follow-up examination for an episode o f dysfunctional uterine bleeding that was successfully treated with a short course of orally administered contraceptives. She had no abdominal o r pelvic pain or dyspareunia. *Associate Professor of Pathology and Gynecology and Obstetrics, Em0ry University School of Medicine, Atlanta. Georgia. "i'Resident in Gynecology and Obstetrics, Emory University School of Medicine, Atlanta. Georgia. ~+Resident in Pathology, Emory University School of Medicine, Atlanta, Georgia. T h e m e n a r c h e was at age 12. Menses p r i o r to the a b n o r m a l bleeding had been regular every 28 days, lasting five days. She had had involunta D' infertility for 11 )'ears. Six years p r i o r to admission she had h a d acute salpingitis, which was treated on an outpatient basis. T h e family history and review o f systems were noncontributory. O n admission the t e m p e r a t u r e was 98.6 ~, tile pulse 84, and the blood pressure 140/I10. T h e patient was 65 inches tall and weighed 190 pounds. T h e r e was no palpable p e r i p h e r al l y m p h a d e n o p a t h y . T h e optic fllndi were unremarkable. T h e a b d o m e n was obese, but a firm n o n t e n d e r mass could be palpated in the right lower quadrant. T h e nmss was smooth and cystic and m e a s u r e d 8 by 10 cm. It was located in the right adnexal area. T h e left adnexa was normal. T h e cervix and vagina were unremarkable. T h e uterus was small, anterior, and mobile. T h e r e m a i n d e r of the physical examination was noncontributory. T h e hernatocrit was 42; the white blood cell count was 7300 with a normal differential. Serum electrolytes, glucose, and blood urea nitrogen levels were normal, and a urinalysis was normal. A l'apanicolaou smear, chest x-ray examination, and kidney-ureter-bladder examination were negative. At laparotomy, n u m e r o u s peritubal, periovarian, and pelvic adhesions were found. T h e r e was a 6 by 10 cm., well circumscribed cystic mass replacing the right ovary. It was moderately a d h e r e n t to the posterior leaf o f the right b r o a d ligament but did not show any evidence o f infiltration into adjacent structures o r an), relationship to the retroperitoneum. T h e right'fallopian tube was densely adh e r e n t to this mass. T h e uterus and left ovary were normal in size and appearance. T h e left fallopian tube was clubbed. T h e r e was no palpable evidence o f metastatic t u m o r in the o m e n t u m or periaortic or pelvic lymph nodes. A right salpingo-oophorectomy with a total resection o f the mass was accomplished without complication. T h e specimen received by laboratory was a large cystic mass measuring 8 by 7 by 2 cm. T h e cut surface exhibited multiloculated cystic spaces filled by a glaD,, viscid, gelatinous material. A rim o f ovarian tissue could be identified at the p e r i p h e r y (Fig. 1). Microscopic examination showed the t u m o r to be composed o f stellate and spindle shaped cells interspersed in loose a b u n d a n t myxoid material (Fig. 2). Tile nuclei were hyperchromatic but no mitoti~c figures were seen. T h e r e was no nuclear pleomorphism. T h e myxoid material stained positively with alcian blue. T h e vascularity o f the t u m o r was variable and consisted o f a few capillary sized vessels without any plexiform vessels being seen. A meshwork o f 723 H U M A N P A T H O L O G Y - - V O L U M E 9, NUMBER 6 November 1978 Figure 1. Cystic ovarian tumor. Ovarian tissue can be ideniified at the top. The tumor shows cystic spaces, partially filled by gelatinous material. delicate reticulum fibers was demonstrated by special staining. Numerous sections failed to reveal an}" other component like cartilage, muscle, or fat. Lipoblasts or embryonal cells were not identified. A special stain for fat was negative. T h e r e was only minimal reactive fibrosis. All sections showed similar histologic features with ovarian tissue identified in some. T h e fallopian tube showed chronic salpingitis and hydrosalpinx. On the basis of these findings the tumor was considered to be a primary ovarian myxoma. T h e patient's postoperative course was uneventful. Pelvic examination was negative 12 months after surgery. DISCUSSION 724 T h e term myxoma is sometimes loosely applied to tumors such as liposarcoma, rhabdomyosarcoma, and chondrosarcoma that only focally contain myxomatous elements. Stout s in 1948 stated the criteria for recogniz!ng myxoma and further re-emphasized them m 1961. 7 According to him, myxoma is a tumor composed of stellate and sometimes spindle shaped cells set in a myxoid stroma containing mucopolysaccharide, through which course very delicate reticulum fibers in vari- ous directions. T h e tumor is usually poorly vascularized, and the capillaries do not have the plexiform arrangement of embryonal lipoblastic tissue. Like any other tumor, it may become fibrosed in some areas. T h e r e must be no chondroblasts, lipoblasts, rhabdomyoblasts, or any other recognizable elements3 In this article the author committed the same error he was warning against? He recognized later that he had included in the group of myxomas several botryoid sarcomas of the genitourinary tracts o f children that were largely myxoid3 It is imperative, therefore, that the criteria for diagnosing myxoma be strictly followed and the more ominous group of sarcomas ~ith myxoid change carefully separated from pure myxomas. Myxomas have been reported to arise from different regions o f the b o d y ? 7 A primary ovarian myxoma in a 14 )'ear old female was first described by Dutz and Stout 7 in 1961. However, this case has not been adequately described and followed. Masubuchi et al. 6 reported another case of ovarian myxoma in 1970. T h e patient was a 43 )'ear old female with a right ovarian mass 9 by 8 by 6 cm. in size. Their gross and microscopic findings are described in detail and provide an acceptable example of ovarian myxoma. T h e patient did MEDICAL INTELLIGENCE am, "~ - ~ I t 9 : .,~ Q ' ~ - " ~ ~ 4h " 4b a ~ ~ a':0 l ' ~ " 4b II; '-i-" 9 11 . i i ' " 9 Figure 2. Microscopic section of the tutnor showing hyperchrotnatic nuclei of stcllate and spindle shaped cells lying in abundant tn) xoid material. (I lcmatoxylin and cosin stain, x 500.) not show any e v i d e n c e o f r e c u r r e n c e o r metastasis about 12 m o n t h s postoperatively. T h e precise histogenesis o f this t u m o r remains a m o o t point. Except in the umbilical c o r d and in o t h e r areas, such as d e n t a l pulp, m y x o m a t o u s tissue d o e s not exist in a d u l t life. T h e r e f o r e , m y x o m a c a n n o t possibly arise as such f r o m this tissue. A c c o r d i n g to Willis, s m y x o m a is a fibroblastic t u m o r that d e v e l o p s substantial a m o u n t s o f intercellular mucin. T h e hyl~otltesis seems u n t e n a b l e , since the t u m o r shows h a r d l y any fibroblastic c o m p o n e n t a p a r t f r o m reactive fibrosis. Besides, o v a r i a n m y x o m a is rare,, w h e r e a s o v a r i a n fib r o m a s are e n c o u n t e r e d frequently. Grossly the t u m o r can be mistaken as a mucinous cystadenoma or pseudomyxoma p e r i t o n e i because o f its cystic n a t u r e a n d m y x o m a t o u s contents. T h e possibility o f a metastatic m u c i n secreting a d e n o c a r c i n o m a s b o u l d be c o n s i d e r e d in all r e l e v a n t c a s e s : It is ext r e m e l y i m p o r t a n t to d i f f e r e n t i a t e b e t w e e n m y x o m a a n d m y x o i d liposarcoma. T h e latter shows m u l t i p l e vascular c h a n n e l s s u r r o u n d e d by small e m b r y o n a l - l i k e ceils, i n c l u d i n g a v a r y i n g n u m b e r o f lipoblasts in which fat can be d e m o n s t r a t e d by special s t a i n s : In spite o f its r e p u t a t i o n for r e c u r r e n c e ) the t u m o r p u r s u e d a m u c h m o r e b e n i g n c o u r s e in a series o f 34 cases r e p o r t e d by Enz i n g e r : a l t h o u g h man)' patients in this g r o u p w e r e treated by only simple excision. A n adeq u a t e surgical excision to include u n i n v o l v e d m a r g i n s is usually r e c o m m e n d e d as a treatm e n t o f choice. References I. Virchow, R.: Die Krankhafieq Geschwfilste, Bd. 1. Berlin, Verlag yon August ltirschwald, 1863, pp. 369-434. 2. Virchow, R.: Die Cellularpathologie in ihrer Begrfindung auf physiologische und pathologiscbe Gewebelehre, .t. Aufl. Berlin, Verlag yon August Hirschwald, 1871, p. 563. 3. Stout, A. P., and Lattes, R.: Tumors of the sol't tissue. Atlas of Tumor Pathology, Fascicle I. Washington, D.C., Armed Forces Institute of Pathology, 1953, pp. 35-38. 4. Enzinger, F. M.: Intramuscular myxoma. Amer, J. Clin. Pathol., 43:104-113, 1965. 5. Stout, A. 1'.: Myxoma, tumor of primitive mesenchyme. Ann. Surg.. 127:706~719, 1948. 6. Masubuchi, K., Kimura, M., Suzumura, H., et al.: Case of ovarian myxoma. Jap. J. Cancer Clin., 16:156-159, 1970. 7. Dutz, W., and Stout, A. P.: The myxoma in childhood. Cancer, 14:629-635, 1961. 8. Willis, R. A.: Pathology of Turnouts. London, Butterworth & Co. (Publishers) Ltd., 1967, pp. 668-669. 725