Acta Chir Belg, 2009, 109, 778-781
Spontaneous Abdominal Wall Endometriosis : a Case Report Th. S. Papavramidis*, K. Sapalidis*, N. Michalopoulos*, G. Karayanopoulou**, G. Raptou**, V. Tzioufa**, I. Kesisoglou*, S. T. Papavramidis* 3rd Department of Surgery*, Department of Pathology**, AHEPA University Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece.
Key words. Endometriosis ; abdominal wall. Abstract. Endometriosis is the presence of endometrial glands and stroma outside the uterus. Spontaneous abdominal wall endometriosis (AWE) is any ectopic endometrium found superficial to the peritoneum without the presence of any previous scar. Rarely, endometriosis represents a disease of specific interest to the general surgeon, on account of its extrapelvic localisations. We describe a case with spontaneous AWE presenting as a painful mass with cyclic symptoms. A 28-year-old woman presented to the day-surgery division of our department, suffering from a painful mass in the left lower abdominal quadrant. A mobile mass of 5 ⫻ 4 cm was identified. The initial diagnosis was lipoma and excision was planned. During the operation two masses were spotted, very close to one another, and were excised within healthy limits. Pathology revealed endometrial glands surrounded by a disintegrating mantle of endometrial stroma and fibrous scar tissue in which there was a scattering of leucocytes. The woman had no scars. She was discharged from hospital after 2 hours. Two years after the excision she is free of disease and no recurrence has been observed. Spontaneous AWE is rare, accounting for 20% of all AWEs. The triad ; mass, pain and cyclic symptomatology helps in the diagnosis, but unfortunately it is not present in all cases. Spontaneous endometriomas are usually diagnosed by pathology and the treatment of choice is surgical excision.
Introduction Endometriosis is defined as the presence of endometrial glands and stroma outside the uterus (1). This ectopic finding occurs in the abdominal wall in 0.03% to 1.08% of women with anamnestic of obstetric or gynaecologic procedures (2). Spontaneous abdominal wall endometriosis (sAWE) is any ectopic endometrium found superficial to the peritoneum without the presence of any previous scar (iatrogenic or not). Rarely, endometriosis represents a disease of specific interest to the general surgeon, on account of its extrapelvic localisations (3). Although some patients present with typical cyclic symptoms associated with their extrapelvic disease, diagnosis can be difficult in those who have symptoms not associated with menstruation. We describe a case with spontaneous AWE presenting as a painful mass with cyclic symptoms. Case report A 28-year-old woman presented to the day-surgery division of our department, suffering from a painful mass in the left lower abdominal quadrant. The mass measured 5 ⫻ 4 cm and was mobile. The initial diagnosis was lipoma of the abdominal wall and excision was planned.
During the operation two masses were spotted, very close to one another, and were excised within healthy limits. The pathology examination revealed endometrial glands surrounded by a disintegrating mantle of endometrial stroma and fibrous scar tissue in which there was a scattering of leucocytes (Fig. 1). The woman had a free gynaecologic and obstetric anamnestic. She was discharged from hospital after 2 hours. Two years after the excision she is free of disease and no recurrence has been observed. Discussion Endometriosis due to ectopic tissue is mainly located in the pelvis, but can also be found at almost any organ of the body, including the lungs, bowel, ureter, brain, and abdominal wall (4, 5). Abdominal wall endometriosis is commonly associated with abdominal surgical scars, especially caesarean section, laparoscopy and amniocentesis (5, 6). Caesarean section is associated with abdominal endometriosis in 0.03-1.7% of all cases (7). Spontaneous AWE is ectopic endometrium found superficial to the peritoneum without previous scars (iatrogenic or not). Spontaneous AWE is less common than scar endometriosis and according to HORTON et al. it represents only 20% of all AWEs (5). However, in the
Abdominal Wall Endometriosis
779
Fig. 1 Endometrial glands surrounded by a disintegrating mantle of endometrial stroma and fibrous scar tissue in which there is a scattering of leucocytes.
bibliography the incidence of spontaneous AWE, in the large series, ranges from 0 to 38% (4, 8-15). The most common place of developing spontaneous AWE is the umbilicus and the groin but there are cases in which the lateral abdominal wall is involved (3, 5, 16). Several theories have been proposed to explain the pathogenesis of endometriosis. The implantation or retrograde menstruation theory was first described by Sampson and proposes that endometrial tissue from the uterus is shed during menstruation and transported retrograde through the fallopian tubes, thereby gaining access to and implanting on pelvic structures (17). The direct transplantation theory is the most probable explanation for endometriosis occurring on scars such as episiotomy, caesarean section, and other scars, whether following surgery or not. The dissemination theory, developed by Halban, proposes endometrial cell migration through vessels (lymphatic and blood vessels) leading to locations outside the pelvis (18, 19). The coelomic metapla-
sia theory hypothesizes that the peritoneal cavity contains progenitor cells or cells capable of differentiating into endometrial tissue (8, 20). This theory is based on embryologic studies demonstrating that all pelvic organs, including the endometrium, are derived from cells lining the coelomic cavity. The induction theory that suggests that sloughed endometrium produces substances to form endometriosis. The embryonic rest theory claiming that a specific stimulus to a Mullerian origin cell nest produces endometriosis. The most recently developed theory is the cellular immunity theory, suggesting that alterations in cell-mediated and humoral immunity allow ectopic endometrial cells to proliferate. In cases where AWE is spontaneous the first two theories, as well as the induction theory, seem incapable of explaining the pathogenesis. However, none of the other theories should be excluded unless experimentation is performed. In AWE, the chief symptom is usually a mass at the site of maximum tenderness (15, 21). This palpable mass
780 usually varies in size following the menstrual cycle. The usual diameter of these masses is between 2 and 3 cm. They have a wide morphologic spectrum, varying from purely cystic chocolate cysts to solid deposits or fibrosis (13, 22, 23). Another characteristic of AWE is cyclic pain associated with menses (9, 22). However, there are studies that describe this pain as constantly present and not associated with the menstrual cycle in the majority of cases (12, 15, 24), but this has generally been regarded as atypical, which may explain why it is often clinically misdiagnosed. Concomitant pelvic endometriosis in cases of endometrioma in a scar was reported in 26% of cases (25). Multiple diagnostic procedures have been used and described. Ultrasound is useful in determining whether a mass is cystic or solid, but the appearance of endometrioma on ultrasound is non-specific (26). CT scan usually shows a solid well-circumscribed mass (27, 28) and may be helpful in showing the extent of the disease. MRI has also been used to diagnose endometriomas (23, 28). FNA is inconclusive but may be of some value in the diagnosis of scar endometriomas (29). Instances of endometrial carcinomas have been reported in these abdominal wall masses (30, 31) and carcinoma or sarcoma of other types may also be present (32). The histological diagnosis of endometrioma requires two of the three following features : endometrial-like glands, endometrial stroma, or hemosiderin pigment. Differential diagnosis of spontaneous AWE, especially when the symptoms are not cyclic, includes abscess, lipoma, haematoma, sebaceous cyst, suture granuloma, inguinal hernia, incisional hernia, desmoid tumour, sarcoma, lymphoma, or primary and metastatic cancer (7). The preferred treatment in cases of abdominal endometriosis is total excision of the mass. If there is assumed or proven pelvic endometriosis, hormonal therapy such as oral contraceptives, Danazol or GnRH analogues can be added to surgical treatment (33). Medical management with gossypol or progesterone may produce only temporary alleviation of the symptoms, with extreme adverse effects followed by recurrence after the cessation of the drugs. Other investigators have also reported poor results with Danazol (23), leuprolide (34), and progesterone (25). The dense and solid scar in the incisions may be nature’s protector for the lesions. In our view, surgical excision is preferable and medical treatment cannot be enthusiastically recommended, except in premenopausal patients. However, special attention has to be paid when excising endometriomas. The excision has to be wide in order to avoid recurrence. Conclusion Spontaneous AWE is rare, accounting for 20% of all AWEs. The triad of mass, pain and cyclic symptomatol-
Th. S. Papavramidis et al. ogy helps in the diagnosis, but unfortunately it is not present in all cases. Spontaneous endometriomas are usually diagnosed by pathology and the treatment of choice is surgical excision.
References 1. OLIVE D. L., SCHWARTZ L. B. Endometriosis. New England Journal of Medicine, 1993, 328 : 1759-1769. 2. CHATTERJEE S. K. Scar endometriosis : a clinicopathologic study of 17 cases. Obstet Gynaecol, 1980, 56 : 81-84. 3. APOSTOLIDIS S., MICHALOPOULOS A., PAPAVRAMIDIS T. S., PAPADOPOULOS V. N., PARAMYTHIOTIS D., HARLAFTIS N. Inguinal endometriosis : Three cases and a literature review. South Med J, 2009, 102 (2) : 206-207. 4. DWIVEDI A. J., AGRAWAL S. N., SILVA Y. J. Abdominal wall endometriomas. Dig Dis Sci, 2002, 47 : 456-461. 5. HORTON J. D., DEZEE K. J., AHNFELDT E. P., WAGNER M. Abdominal wall endometriosis : a surgeon’s perspective and review of 445 cases. Am J Surg, 2008, 196 : 207-212. 6. TOMÁS E., MARTÍN A., GARFIA C., SÁNCHEZ GÓMEZ F., MORILLAS J. D., CASTELLANO TORTAJADA G., SOLÍS HERRUZO J. A. Abdominal wall endometriosis in the absence of previous surgery. J Ultrasound Med, 1999 May, 18 (5) : 373-374. 7. SEYDEL A. S., SICKEL J. Z., WARNER E. D., SAX H. C. Extrapelvic endometriosis : diagnosis and treatment. Am J Surg, 1996, 171 : 239. 8. STECK W. D., HELWIG E. B. Cutaneous endometriosis. Clin Obstet Gynaecol, 1966, 9 : 373-383. 9. SINGH K. K., LESSELLS A. M., ADAM D. J., JORDAN C., MILES W. F., MACINTYRE I. M., GREIG J. D. Presentation of endometriosis to general surgeons : a 10-year experience. Br J Surg, 1995, 82 : 13491351. 10. SCHOLEFIELD H. J., SAJJAD Y., MORGAN P. R. Cutaneous endometriosis and its association with caesarean section and gynaecological procedures. J Obstet Gynaecol, 2002, 22 : 553-554. 11. DOUGLAS C., ROTIMI O. Extra-genital endometriosis – a clinicopathological review of a Glasgow hospital experience with case illustrations. J Obstet Gynaecol, 2004, 24 : 804-808. 12. BLANCO R. G., PARITHIVEL V. S., SHAH A. K., GUMBS M. A., SCHEIN M., GERST P. H. Abdominal wall endometriomas. Am J Surg, 2003, 185 : 596-598. 13. FRANCICA G., GIARDIELLO C., ANGELONE G., CRISTIANO S., FINELLI R., TRAMONTANO G. Abdominal wall endometriomas near caesarean delivery scars : sonographic and colour doppler findings in a series of 12 patients. J Ultrasound Med, 2003, 22 : 1041-1047. 14. GUNES M., KAYIKCIOGLU F., OZTURKOGLU E., HABERAL A. Incisional endometriosis after caesarean section, episiotomy and other gynaecologic procedures. J Obstet Gynaecol Res, 2005, 31 : 471475. 15. HENSEN J. H., VAN BREDA VRIESMAN A. C., PUYLAERT J. B. Abdominal wall endometriosis : clinical presentation and imaging features with emphasis on sonography. AJR Am J Roentgenol, 2006, 186 : 616-620. 16. MILLER W. B., MELSON G. L. Abdominal wall endometrioma. AJR Am J Roentgenol, 1979, 132 : 467-468. 17. SELI E., BERKKANOGLU M., ARICI A. Pathogenesis of endometriosis. Obstet Gynaecol Clin North Am, 2003, 30 : 41-61. 18. ICHIMIYA M., HIROTA T., MUTO M. Intra-lymphatic embolic cells with cutaneous endometriosis in the umbilicus. J Dermatol, 1998, 25 : 333-336. 19. HALBAN J. Metastatic hystero-adenosis. Wien Klin Wochenschr, 1924, 37 : 1205-1206. 20. MATSUURA K.., OHTAKE H., KATABUCHI H., OKAMURA H. Coelomic metaplasia theory of endometriosis : evidence from in vivo studies and an in vitro experimental model. Gynaecol Obstet Invest, 1999, 27 : 18-20. 21. KHAMMASH M. R., OMARI A. K., GASAIMEH G. R., BANI-HANI K. E. Abdominal wall endometriosis. An overlooked diagnosis. Saudi Med J, 2003, 24 : 523-525.
781
Abdominal Wall Endometriosis 22. WOODWARD P. J., SOHAEY R., MEZZETTI T. P. Endometriosis : radiologic-pathologic correlation. RadioGraphics, 2001, 21 : 193216. 23. WOLF C., OBRIST P., ENSINGER C. Sonographic features of abdominal wall endometriosis. AJR, 1997, 169 : 916-917. 24. KOGER K. E., SHATNEY C. H., HODGE K., MCCLENATHAN J. H. Surgical scar endometrioma. Surg Gynecol Obstet, 1993, 177 : 243-246. 25. RANI P. R., SOUNDARARAGHAVAN S., RAJARAM P. Endometriosis in abdominal scars – a review of 27 cases. Int J Gynaecol Obstet, 1991, 36 : 215-218. 26. VINCENT L. M., MITTELSTRADET C. A. Sonographic demonstration of endometrioma arising in a caesarean section scar. J Ultrasound Med, 1985, 4 : 437-438. 27. AMATO M., LEVITT R. Abdominal wall endometrioma : CT findings. J Comput Assisted Tomogr, 1984, 8 : 1213-1214. 28. CHIN Y. Y., PEREZ-REYES M., BROWN J. J. MR appearance of umbilical endometriosis. J Comput Assist Tomogr, 1994, 18 : 269271. 29. GRIFFIN J. B., BETSILL W. L. Subcutaneous endometriosis diagnosed by fine needle aspiration cytology. Acta Cytol, 1985, 29 : 584-588.
30. MADSEN H., HANSEN P., ANDERSON O. P. Case report : endometrioid carcinoma in an operation scar. Acta Obstet Gynaecol Scand, 1980, 59 : 475-476. 31. KOTWALL C. A., KIRKBRIDE P., ZERAFA A. E., MURRAY D. Endometrial cancer and abdominal wound recurrence. Gynaecol Oncol, 1994, 53 : 357-360. 32. KENNEDY S., BRODRIBB J., GODFREY A. M., BARLOW D. H. Preoperative treatment of an abdominal wall endometrioma with nafarelin acetate. Br J Obstet Gynaecol, 1988, 95 : 521-523. 33. CHUN J. T., NELSON H. S., MAULL Kl. Endometriosis of the abdominal wall. South Med J, 1990, 83 : 1491-1492. 34. PURVIS R. S., TYRING S. K. Cutaneous and subcutaneous endometriosis. Surgical and hormonal therapy. J Dermatol Surg Oncol, 1994, 20 : 693-695.
Theodossis S. Papavramidis, M.D., Ph.D. 30 Korytsas str., Panorama 55236 Thessaloniki, Greece Tel. : 00306944536972 E-mail :
[email protected]
