Spontaneous Endometriosis of the Abdominal Wall

Endometriosis is characterized by the abnormal growth of endometrial tissue outside the uterine cavity. Most endometrial deposits are found in the pelvis including ovaries, peritoneum, uterosacral ligaments, pouch of Douglas, and rectovaginal septum, but it may rarely oc- cur in extra pelvic locations. These include most body cavities, as well as organs including lung, gallbladder, bowel, kidney, central nerv- ous system, extremities, perineum, and abdominal wall (1-4). Extrapelvic endometriosis may occur in up to 12 percent of women with endometriosis (5). Umbilical endometriosis is rare with an estimated incidence of 0.5 to 1.0 percent of all patients with endometrial ectopia.6 More commonly, cutaneous endometriosis occurs in a surgical scar from abdominal or pelvic procedures, which include hysterectomy, cesarean sections, episiot- omy and laparoscopy (7,8). As endometriosis is rarely seen by general surgeons and is often diagnosed on his- tological examination postoperatively due to a large number of potential pitfalls in its diagnosis, we report 3 cases ,two of them with a diagnosis of unusual devel- opment of umbilical endometriosis and one with ingui- nal endometriosis, from the perspective of a general sur- geon. Case reports Case 1 A 40-year-old woman was referred to our general surgery clinic due to a five month history of bleeding from her umbilicus which was concurrent with her men- struation period. In her past medical history, she had the diagnosis of uterine fibroma due to the mid cycle spot- ting from 4 years ago. She had the history of 3 Natural vaginal deliveries. She had no history of pain or previ- ous caesarean section or any other surgery. Ultrasono- graphy (USG) revealed a myomatosis uterus with a right subserosal myoma. She underwent abdominal hysterec- tomy and left oophorectomy. The uterus had a big sub- serosal myoma in the left fundus and two intramural myomas in the corpus of the uterus. It was also a 1×0.5 cm mass in the umbilicus, which was totally excised. It was confirmed to be endometriosis with histopathology. She was discharged from the hospital after 1 day without any complication. Case 2 A 37-year-old woman presented with a blue-violet um- bilical nodule with bloody and pussy discharge. She had gynecological visit the previous year for the abnormal menstruation and spotting with pain and abdominal mass sensation. USG revealed a 9x5 cm cystic lesion in the right adnexa and a multiseptate cystic lesion in the left. It also revealed a solid lesion accompanied by a small cystic part which was extended from the right adnexa to the abdominal wall which was suspected to be a uterine fibroma. During the surgery with sub and circumumbili- cal incision, fibromatous uterus was detected while two big and subserosal fibromas were excised. The ovaries were polycystic which the big ones were undergone cys- tectomy. There were also two prominent mass (ap- proximately 1×0.5 cm) in the umbilicus which were to- www.SID.ir Archive of SID L. Geranpayeh, et al. Acta Medica Iranica, Vol. 47, No. 2 (2009) 155 tally excised. The pathologic report from the ovaries and umbilical masses was respectively endometriotic cysts and cutaneous endometriosis. She had bloody and pussy discharge from the umbilicus after 5 months which ac- companied with pain and discomfort. It was revealed a 2x2 painful black mass on examination with the above- mentioned characteristics. The patient again underwent a surgery and the aforesaid mass which contained granula- tion tissue and hair was entirely excised. It was extended up to the peritoneum. The pathology reported endome- triosis. She was discharged from hospital after 5 days without any complication. Case 3 A 38-year-old woman was referred to the surgical clinic due to a painful inguinal mass. She had pain and dis- comfort in the right inguinal region for about a year. She underwent a surgery with the preoperative diagnosis of irreducible sliding hernia. There was no history of any prior surgery or caesarian section. During the surgery a mass with bloody discharge was detected on the medial side of the indirect hernia sac. The hernia sac was ex- cised with mentioned mass and the pathology report revealed endometriosis of the hernia sac. She was dis- charged from hospital after 2 days without any compli- cation.

Different pathophysiological theories concerning the origins of endometriosis have been proposed, including the implantation or reflux, direct extension, coelomic metaplasia (claiming that endometriosis develops from metaplasia of peritoneum), induction (suggesting that sloughed endometrium results in endometriosis), em- bryonic rest (claiming a specific stimulus to a Mullerian origin cell nest produces endometriosis) and lymphatic and vascular metastasis (9). But we didn’t recognize a net etiology for our three cases. The diagnosis of extra genital endometriosis could be difficult due partly to a low index of suspicion by both general practitioners and specialists. Endometriosis of the skin is a well- recognized pathologic entity usually encountered clini- cally as a periumbilical lesion or within abdominal sur- gical scars after cesarean section or other gynecologic procedures (10). Umbilical endometriosis may also de- velop in the surgical scar after abdominal procedures or the trocar site of laparoscopic procedures, but it may rarely occur spontaneously in the physiologic scar of the umbilicus (11), while our first two cases had this kind of endometriosis. Inguinal endometriosis is also rare and may be difficult to recognize. Often it is confused with other more common disorders of the groin, such as lym- phadenopathy, hernia, granuloma, neuroma, abscess, lipoma, hematoma, soft-tissue tumor, metastatic cancer and subcutaneous cyst. The majority of cases are be- lieved to be caused by progression of pelvic endometrio- sis down the round ligament into the inguinal canal. In- guinal endometriosis has been described after gyneco- logic surgery, and only a minority of cases has been as- sociated with a hernial sac (12), such as our third pa- tient. She had no history of any prior surgery or caesar- ian section. Although the reasons for right-sided pre- dominance have not been identified, over 90% of ingui- nal endometriosis occurs on the right (13) and the disor- der occurred on the right side of our case too. This case may be of interest to the general surgeon who commonly manages patients with groin masses but does not deal often with endometriosis. Hernias associated with en- dometriosis may not be clinically detectable. even though no proven explanation can be offered; the scar- ring reaction that surrounds endometrial foci may reduce tissue elasticity, thus hindering hernia detection on physical examination (14). A blue-violet painful nodule with symptoms of bleeding or discharge synchronous with the menstrual cycle is pathognomonic for endometriosis. Only one (case 1) of our patients had these characteristics. Pain was a remarkable complaint in two (case 2 and 3) of our patients, but they didn’t have a cyclic pain which could help in establishing the diagnosis. A preoperative ultra- sonographic examination has helped to determine the size of the lesion and whether the mass is cystic or solid and has been useful in excluding underlying intra- abdominal pathologic factors (15,16). But the reported appearance of endometriosis on ultrasonographic ex- amination is nonspecific and may change during the course of a menstrual cycle (17-19). Altogether ultra- sonography should be applied preoperatively in a suspi- cious case of endometriosis. We had also applied USG for all our three cases. Computed tomographic scans and magnetic resonance imaging are also useful in ruling out incisional hernias and showing a direct association of the mass and the abdominal wall, but they do not pro- vide a definitive preoperative diagnosis (15,16,20), we didn’t use these techniques preoperatively. Fine-needle aspiration biopsy had been reported in some studies to help confirm the diagnosis and effectively eliminate the possibility of malignancy (16,21), but in other studies it has been reported of inconclusive help or needless (20,22), or even to increase the risk of recurrence (23). A large retrospective study has concluded that fine- www.SID.ir Archive of SID Spontaneous Endometriosis 156 Acta Medica Iranica, Vol. 47, No. 2 (2009) needle aspiration (FNA) or percutaneous biopsy should not be suggested when endometriosis is suspected (24), so we did not perform FNA in our patients. Simple sur- gical excision of the endometriosis is the treatment of choice, with sparing of the umbilicus when possible, in the case of umbilical endometriosis (25) Local recur- rence after adequate surgical excision is uncommon. Malignancy of ectopic endometrial tissue has also been reported (26). Thus wide excision of endometriosis in the present cases enabled us to gain clear surgical margins to avoid leaving the rest of the ectopic tissue. The possibility of coexisting pelvic endometriosis should be investigated by Postoperative follow-up with a gynecologist. Endometriosis should be considered in any woman of childbearing age with a painful or tender ab- dominal mass. Imaging studies such as ultrasound, CT or MRI are non-specific; thus, a biopsy is necessary to make a definitive diagnosis. Endometriosis should be considered in the differential diagnosis of abdominal wall lesions even if not associated with a scar of a previ- ous operation. Biopsy is diagnostic and surgical excision is curative (27). In conclusion, although there are very few cases reported in the literature in whom a surgical history was absent (13), all our three patients had this unique characteristic. And since it is often diagnosed only upon histological examination postoperatively, it should be emphasized in the differential diagnosis of abdominal wall masses.

1. Steck WD, Helwig EB. Cutaneous endometriosis. Clin Obstet Gynecol 1966; 9:373-383. 2. Franklin RR, Navarro C. Extragenital endometriosis. Prog Clin Biol Res 1990; 323:289-295. 3. Yu CH, Perez-Reyes M, Brown JJ, Borrello JA. MR ap- pearance of umbilical endometriosis. J Comput Assist To- mogr 1994; 18:267-271. 4. Sataloff DM, La Vorgna KA, McFarland MM. Extrapelvic endometriosis presenting as a hernia: clinical reports and review of the literature. Surgery 1989; 105:109-112. 5. Franklin RR, Navarro C. Extragenital endometriosis. In: Chadha DR, Buttran VC, eds. Current Concepts in Endo- metriosis. New York: Alan R. Liss, Inc. 1990, p. 289. 6. Michowitz M, Baratz M, Stavorovsky M. Endometriosis of the umbilicus. Dermatologica 1983; 167(6):326-330. 7. Steck WD. Cutaneous endometriosis. JAMA 1965; 191:167. 8. Albrecht LE, Tron V, Rivers JK. Cutaneous endometriosis. International Journal of Dermatology 1995 Apr; 34(4):261- 262. 9. Witz CA. Current concepts in the pathogenesis of endome- triosis. Clin Obstet Gynecol 1999; 42:566-585. 10. Fair KP, Patterson JW, Murphy RJ, Rudd RJ. Cutaneous deciduosis. J Am Acad Dermatol 2000; 43:102-107. 11. Majeski J, Craggie J. Scar Endometriosis Developing After an Umbilical Hernia Repair with Mesh. Southern Medical Association 2004; 97:532-534. 12. Brzezinski A, Durst AL. Endometriosis presenting as an inguinal hernia. AmJ Obstet Gynecol 1983; 146:982-983. 13. Candiani GB, Vercellini P, Fedele L,Nicoletta V, Carinelli S, Scagkione V. Inguinalendometriosis: pathogenetic and clinical implications. Obstet Gynecol 1991; 78:191-194. 14. Boggi U, Del Chiaro M, Pietrabissa A, Mosca F. Extrapelvic endometriosis associated with occult groin hernias. Canadian Medical Association 2001; 44(3):224. 15. Yu CY, Perez-Reyes M, Brown JJ, Borrello JA. MR ap- pearance of umbilical endometriosis. J Comput Assist To- mogr 1994; 18:267-271. 16. Wolf GC, Singh KB. Cesarean scar endometriosis: a review. Obstet Gynecol Surv 1989; 44:89-95. 17. Patterson GK, Winburn GB. Abdominal wall endometrio- mas: report of eight cases. Am Surgeon 1999; 65:36-39. 18. Alexiadis G, Lambropoulou M, Deftereos S, Giatromanolaki A, Sivridis E, Manavis J. Abdominal wall endometriosis: ultrasound research: a diagnostic problem. Clin Exp Obstet Gynecol 2001; 28:121-122. 19. Nirula R, Greaney GC. Incisional endometriosis: an under- appreciated diagnosis in general surgery. J Am Coll Surg 2000; 190:404-407. 20. Dwivedi AJ, Agrawal SN, Silva YJ. Abdominal wall endometriomas. Dig Dis Sci 2002; 47:456-4561. 21. Simsir A, Thorner K, Waisman J, Cangiarella J. Endome- triosis in abdominal scars: a report of three cases diagnosed by fine-needle aspiration biopsy. Am Surgeon 2001; 67:984-986. 22. Koger KE, Shatney CH, Hodge K, McClenathan JH. Surgical scar endometrioma. Surg Gynecol Obstet 1993; 177:243-246. 23. Matthes G, Zabel DD, Nastala CL, Shestak KC. Endo- metrioma of the abdominal wall following combined ab- dominoplasty and hysterectomy: case report and review of the literature. Ann Plast Surg 1998; 40:672-675. 24. Zhao X, Lang J, Leng J, Liu Z, Sun D, Zhu L. Abdominal wall endometriomas. International Journal of Gynecology and Obstetrics 2005; 90:218-222. 25. Purvis RS, Tyring SK.Cutaneous and subcutaneous endo- metriosis surgical and hormonal therapy. Journal of Derma- tologic Surgery and Oncology 1994 Oct; 20(10):693-695. 26. Hitti IF,Glasberg SS,Lubicz S. Clear cell carcinoma arising in extraovarian endometriosis: report of three cases and review of the literature. Gynecol Oncol 1990; 39:314-320. 27. Ideyi SC, Schein M, Niazi M, Gerst PH. Spontaneous Endometriosis of the Abdominal Wall. Digestive Surgery 2003; 20:246-248. www.SID.ir