Endometrioid Adenocarcinoma in an Extrauterine Adenomyoma Michael A. Ulm, MD, MS, David B. Robins, MD, Edwin M. Thorpe Jr, MD, and Mark E. Reed, MD BACKGROUND: Focal involvement by endometrioid adenocarcinoma in an extrauterine adenomyoma in a patient with stage 1 endometrioid adenocarcinoma presented a unique problem in staging and management of extrauterine endometrial cancer. CASE: A 49-year-old white woman, gravida 0, referred for endometrioid adenocarcinoma was found to have an extrauterine adenomyoma involved with endometrioid adenocarcinoma in the inguinal canal after surgical staging. The endometrioid adenocarcinoma involving the extrauterine adenomyoma was low-grade and noninvasive, representing an embryological anomaly transformed into endometrioid adenocarcinoma by unopposed estrogen. Stage 1A, grade 2 endometrioid adenocarcinoma was diagnosed and observed. CONCLUSION: Stage 1 endometrioid adenocarcinoma with concurrent, noninvasive, focal involvement in an extrauterine adenomyoma represents a secondary site and does not alter disease stage. (Obstet Gynecol 2014;124:445–8) DOI: 10.1097/AOG.0000000000000391
E
ndometrial adenocarcinoma is the most common gynecologic cancer in the United States, with more than 50,000 new cases and almost 8,600 deaths each year.1 Type I endometrioid adenocarcinoma represents approximately 80% of endometrial cancers and arises in the setting of continuous estrogen stimulation. Although common, the prognosis of endometrioid adenocarcinoma is generally favorable because approximately 75% of cases are diagnosed while confined to the uterine corpus. Factors associated From the Departments of Obstetrics and Gynecology and Pathology and Laboratory Medicine, University of Tennessee Health Science Center, and Department of Obstetrics and Gynecology, University of Tennessee Health Science Center—West Clinic, Memphis, Tennessee. Corresponding author: Michael A. Ulm, MD, MS, University of Tennessee Health Science Center, Department of Obstetrics and Gynecology, 853 Jefferson Avenue, Room E102, Memphis, TN 38163; e-mail: [email protected]. Financial Disclosure The authors did not report any potential conflicts of interest. © 2014 by The American College of Obstetricians and Gynecologists. Published by Lippincott Williams & Wilkins. ISSN: 0029-7844/14
VOL. 124, NO. 2, PART 2, AUGUST 2014
with clinical outcome include histologic grade, depth of myometrial invasion, lymphovascular space involvement, and lymph node involvement.2 Adenomyosis is present in 10% to 70% of hysterectomy specimens and is secondarily involved with endometrioid adenocarcinoma approximately 21% to 30% of the time when both conditions are present.3 Including our patient, 36 cases of extrauterine adenomyomas have been reported, and they are typically found in the adnexa.4 Endometrioid adenocarcinoma in an extrauterine adenomyoma found in our patient presents an interesting challenge in staging and management of extrauterine disease not attributed to metastasis from the uterine corpus. Our discussion encompasses the etiology of the lesion as well as current literature and recommendations regarding staging of endometrioid adenocarcinoma involving an extrauterine adenomyoma.
CASE A 49-year-old woman, gravida 0, with a 10-year history of metomenorrhagia presented to her gynecologist after being hospitalized for vaginal bleeding and significant anemia requiring blood transfusion. Endometrial biopsy was performed, with findings of a grade 2 endometrioid adenocarcinoma, and she was referred to a gynecologic oncologist. The patient’s medical history was significant for morbid obesity, chronic anemia, and hypertension. Her hypertension was controlled with multiple antihypertensive medications. She had undergone dilation and curettage for menorrhagia with benign pathology in 1983. She experienced menarche at age 9 years and had a history of regular menses until the past 10 years. She had not previously undergone evaluation for her metomenorrhagia and she had not undergone gynecologic evaluation in more than 15 years. She denied any history of contraceptive use or ever having sexual intercourse. On presentation, her vital signs were within normal limits (weight 240.4 lb, height 63 inches, and body mass index 42.6). She had a visually normal cervix and vagina with minimal bleeding. On examination, her uterus was estimated to be the size of that of a woman at 16 weeks of gestation, and it was compromised by a morbidly obese abdomen. A 3-cm firm mass suspicious for inguinal adenopathy was palpated in her left inguinal region. On questioning, the patient stated the inguinal mass had been palpable for an unknown number of years. Total abdominal hysterectomy, bilateral salpingo-oophorectomy, periaortic and pelvic lymph node dissection, and left inguinal lymph node dissection were surgically staged. During the left inguinal lymph node dissection, it was clear the mass was not arising from the lymph nodes but rather from the left round ligament, and it extended under the inguinal ligament into the left femoral triangle. Intraoperatively, gross section of the left inguinal mass was suspicious for metastatic disease. The right round ligament was grossly
Ulm et al
Carcinoma in an Extrauterine Adenomyoma
445
Fig. 1. Pathologic section from the extrauterine adenomyoma with foci of endometrioid adenocarcinoma surrounded by endometrial stroma and myometrium. Ulm. Carcinoma in an Extrauterine Adenomyoma. Obstet Gynecol 2014.
normal, and suture was ligated in the standard manner. The patient had an uncomplicated hospital course. Final pathology was International Federation of Gynecology and Obstetrics (FIGO) stage 1A, grade 2. The intrauterine mass was 11.5 cm at its greatest dimension, with 1.7 cm myometrial invasion or 45% of the myometrium, and without lymphatic or vascular space invasion. The uterine corpus had focal adenomyosis without endometrioid adenocarcinoma involvement. The fallopian tubes, ovaries, cervix, and lymph nodes were negative for malignancy. The pathology report of the tissue submitted as “left inguinal lymph node” contained a 3.8-cm
prominent smooth muscle nodule containing many endometrial glands demonstrating foci of adenocarcinoma. The glandular proliferation was contained within the smooth muscle, without infiltration into the soft tissue surrounding the smooth muscle (Fig. 1). The smooth muscle nodule and endometrial stroma within it were positive for desmin and CD10 immunostains, respectively (Fig. 2). Immunohistochemistry was positive for estrogen and progesterone receptors in 90 to 100% of the tumor cells in both the uterus and extrauterine adenomyoma. The constellation of morphologic and immunohistochemical findings were consistent with an extrauterine adenomyoma, focally involved with endometrioid adenocarcinoma, in the left round ligament. The findings were presented at Tumor Board, a multidisciplinary conference involving pathology, gynecologic oncology, radiation oncology, and interventional radiology. It was agreed that the extrauterine adenomyoma contained a second primary site of endometrioid adenocarcinoma and did not represent metastatic disease. The recommendation was made for follow-up every 3 months, with a computed tomography scan of the abdomen and pelvis every 6 months for the first 2 years. At her 6-month followup, she was doing well without evidence of disease.
COMMENT We performed a review of the literature using the MEDLINE-Ovid database. Our search was in the English language from 1950 to 2014; the search included the terms “extrauterine adenomyoma,” “uterus-like,” “adenomyoma cancer,” “adenomyoma carcinoma,” and “adenomyoma adenocarcinoma.” Including this patient, there are 36 cases of
Fig. 2. Pathologic section from the extrauterine adenomyoma with foci of endometrioid adnocarcinoma. A. Hematoxylin and eosin stain. B. Desmin stain for myometria stroma. C. CD10 stain for endometrial stroma. Both the desmin and CD10 stains show the glandular hyperplasia completely surrounded by myometrium and endometrial stroma, respectively. Ulm. Carcinoma in an Extrauterine Adenomyoma. Obstet Gynecol 2014.
446
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Carcinoma in an Extrauterine Adenomyoma
OBSTETRICS & GYNECOLOGY
extrauterine adenomyomas, or uterus-like masses, in the English literature (Table 1). Including our case, only two malignant uterus-like masses or extrauterine adenomyomas have been reported in the literature. Other reports have detailed masses ranging from 1 to 20 cm in size, with cystic components, adenomyosis, or large cavities lined with endometrial tissue.4–19 However, this is the first report of concurrent involvement of endometrioid adenocarcinoma in both an extrauterine adenomyoma and uterine fundus. Adenomyosis is defined as the intramyometrial presence of endometrial mucosa surrounded by reactive, hypertrophic myometrium. The etiology of adenomyosis is unknown. Endometrium and adjacent myometrium have a common embryological origin from the Mullerian ducts. The most often quoted hypothesis for the origin of adenomyosis is invagination of endometrial tissue between the smooth muscle fibers of the myometrium. This case, however, lends credence to the theory of adenomysosis formation from metaplasia of de novo ectopic endometrial tissue during embryologic development.20 During Mullerian duct differentiation, the female gubernaculum is formed from the caudal fold of the Wolffian body, which is provoked by the mesonephros elevating the covering peritoneum. It extends from the gonadal ridge to the future inguinal region and is composed of mesenchymal cells and muscular and extracellular matrix precursors. Although the origin of the female gubernaculum is controversial, it is generally accepted that the cranial portion forms the utero-ovarian ligament and the caudal portion forms the round ligament. Tumors in the inguinal canal involving the round ligament are rare and are typically leiomomyomas. Most published cases are associated with Mullerian agenesis and Rokitansky syndrome, forming indirect hernias.21 Although this patient did not have renal malformation, it is plausible to suggest that a portion of her Mullerian duct migrated along the urogenital ridge to form the extrauterine adenomyoma during embryologic development of the genital tract. Unopposed estrogen, in the setting of chronic anovulation, produced in adipose tissue has a mitogenic effect on endometrial cells.22 The majority of cases of endometrioid adenocarcinoma result from estrogenic stimulation of endometrial hyperplasia or endometrial intraepithelial neoplasia as a precursor lesion.2 We contend that unopposed estrogen is the cause of the uterine endometrioid adenocarcinoma and the endometrioid adenocarcinoma contained within the existing extrauterine adenomyoma.
VOL. 124, NO. 2, PART 2, AUGUST 2014
Table 1. Locations and Frequency of Sites of the 36 Extrauterine Adenomyomas Reported in the Literature Site
n
%
Ovary Broad ligament Abdomino-pelvic Pelvic wall Small bowel mesentary Conus medullaris Ovarian ligament Colon Inguinal canal Ileum Round ligament Obturator lymph node Posterior cul-de-sac Appendix
10 5 4 3 2 2 2 2 1 1 1 1 1 1
27.7 13.8 11.1 8.3 5.6 5.6 5.6 5.6 2.8 2.8 2.8 2.8 2.8 2.8
Data from references 4–19.
Hanley et al3 performed a retrospective analysis of 82 hysterectomy specimens with adenomyosis involved with endometrioid adenocarcinoma invading less than 50% of the myometrium (FIGO stage 1A). In otherwise low-stage tumors, they concluded that endometrioid adenocarcinoma involved in deeply located adenomyosis does not affect prognosis. They further state “myometrial-based foci of well-differentiated endometrioid adenocarcinoma, completely or partially surrounded by endometrial stroma, most likely represents tumor colonized adenomyosis.”3 According to the FIGO staging system for endometrial cancers, all metastatic disease located outside of the peritoneal cavity is stage IVB.2 In this case, the lesion was well-differentiated, completely surrounded by endometrial stroma, and completely resected from the inguinal canal. The extrauterine adenomyoma with focal endometrioid adenocarcinoma is a second primary site of endometrioid adenocarcinoma and not metastatic disease outside of the peritoneal cavity. The findings support the final diagnosis of FIGO stage 1A, grade 2 endometrioid adenocarcinoma. REFERENCES 1. Siegal R, Ma J, Zou Z, Jemal A. Cancer statistics, 2014. CA Cancer J Clin 2014;64:9–29. 2. Sorosky J. Endometrial cancer. Obstet Gynecol 2012;120: 383–97. 3. Hanley K, Dustin M, Stoler M, Atkins K. The significance of tumor involved adenomyosis in otherwise low-stage endometrioid adenocarcinoma. Int J Gynecol Pathol 2010;29:445–51. 4. Carinelli S, Motta F, Frontino G, Restelli E, Fedele L. Multiple extrauterine adenomyomas and uterine-like masses: case reports and review of the literature. Fertil Steril 2009;91:1956.e9–11.
Ulm et al
Carcinoma in an Extrauterine Adenomyoma
447
5. Rahilly MA, al-Nafussi A. Uterus-like mass if the ovary associated with endometrioid carcinoma. Histopathology 1991;18: 549–51. 6. Bulut AS, Sipahi TU. Abscessed uterine and extrauterine adenomyomas with uterus-like features in a 56-year-old woman. Case Rep Obstet Gynecol 2013;2013:238156. 7. Moghadamfalahi M, Metzinger DS. Multiple extrauterine adenomyomas presenting in upper abdomen and pelvis: a case report and brief review of the literature. Case Rep Obstet Gynecol 2013;2102:565901. 8. Sisodia SM, Khan WA, Goel A. Ovarian ligament adenomyoma: report of a rare entity with review of the literature. J Obstet Gynaecol Res 2012;38:724–8. 9. Khurana A, Mehta A, Sardana M. Extrauterine adenomyoma with uterus like features: a rare entity presenting 17 years posthysterectomy. Indian J Pathol Microbiol 2011;54:572–3. 10. Stewart CJ, Leung YC, Mathew R, McCartney AL. Extrauterine adenomyoma with atypical (Symplastic) smooth muscle cells: a report of 2 cases. Int J Gynecol Pathol 2009;28:23–8. 11. Kim JO, Baek JM, Jeung C, Park EK, Lee HN, Lee YS. A case of primary ovarian adenomyoma mimicking ovarian malignancy. Eur J Gynaecol Oncol 2011;32:103–6. 12. Bayar U, Demirtas E, Usubutun A, Basaran M, Esinler I, Yarali H. Ovarian adenomyoma following gonadotropin treatment for infertility. Reprod Biomed Online 2006;13:676–9. 13. Nechi S, Znaidi N, Rammah S, M’farej MK, Zermani R. Uterus-like mass of the broad ligament. Int J Gynaecol Obstet 2013;123:249–50. 14. Sharma MC, Sarkar C, Jain D, Suri V, Garg A, Vaishya S. Uterus-like mass of mullerian origin in the lumbosacral region
Endometrial Adenocarcinoma Presenting as a Hematotrachelos Christopher M. Sauer, MD, MBA, Sudeshna Chatterjee, MD, Gary M. Israel, MD, and Peter E. Schwartz, MD BACKGROUND: Hematotrachelos, distension of the uterine cervix with accumulated blood, is an extremely rare condition resulting from a congenital anomaly or an acquired condition. We present a case in which an
causing cord tethering. Report of two cases. J Neurosurg Spine 2007;6:73–6. 15. Seki A, Maeshima A, Nakaqawa H, Shiraishi J, Murata Y, Arai H, et al. A subserosal uterus-like mass presenting after a sliding hernia of the ovary and endometriosis: a rare entity with a discussion of histogenesis. Fertil Steril 2011;95:1788.e15–19. 16. Shin SY, Kim HJ, Kim YW, Lee KY. CT characteristics of a uterus-like mass in the sigmoid mesocolon. Br J Radiol 2011;84:e1–3. 17. Carvalho FM, Carvalho JP, Pereira RM, Ceccato BP Jr, Lacordia R, Baracat EC. Leiomyomatosis peritonealis disseminata associated with endometriosis and multiple uterus-like masses: report of two cases. Clin Med Insights 2012;5:63–8. 18. Liang YJ, Hao Q, Wu YZ, Wu B. Uterus-like mass in the left broad ligament misdiagnosed as a malformation of the uterus: a case report of a rare condition and review of the literature. Fertil Steril 2010;93:1347.e13–6. 19. Na K, Kim GY, Won KY, Kim HS, Kim SW, Lee CH, et al. Extrapelvic uterus-like masses presenting as colonic submucosal tumor: a case study and review of literature. Korean J Pathol 2013;47:177–81. 20. Acien P, Sanchez del Campo F, Mayol MJ, Acien M. The female gubernaculum: role in the embryology and development of the genital tract and in the possible genesis of malformations. Eur J Obstet Gynecol Reprod Biol 2011;159:426–32. 21. Ferenczy A. Pathophysiology of adenomyosis. Hum Reprod Update 1998;4:312–22. 22. Lindemann K, Vatten LJ, Ellstrom-Engh M, Eskild A. Body mass, diabetes and smoking, and endometrial cancer risk: a follow-up study. Br J Cancer 2008;98:1582–5.
acquired hematotrachelos was the presenting sign of endometrial cancer. CASE: An asymptomatic 66-year-old woman was found to have a bulging cervix during a well-woman visit. Further workup revealed a hematotrachelos and an underlying endometrial adenocarcinoma. She was treated with surgery and adjuvant radiotherapy. CONCLUSION: A hematotrachelos, although rare, can prevent vaginal bleeding, which is often the earliest symptom of a uterine malignancy. This case report illustrates the potential importance of the pelvic examination as part of the well-woman physical examination, because it led to the discovery of early-stage endometrial cancer. (Obstet Gynecol 2014;124:448–51)
From the Departments of Obstetrics, Gynecology, and Reproductive Sciences and Diagnostic Radiology, Yale University School of Medicine, New Haven, Connecticut. Corresponding author: Christopher M. Sauer, BA, 123 York Street, Apt 12A, New Haven, CT 06511; e-mail: [email protected]. Financial Disclosure The authors did not report any potential conflicts of interest. © 2014 by The American College of Obstetricians and Gynecologists. Published by Lippincott Williams & Wilkins. ISSN: 0029-7844/14
448
Sauer et al
Hematotrachelos
DOI: 10.1097/AOG.0000000000000281
H
ematotrachelos is a rare condition in which the uterine cervix becomes distended with blood, resulting from a congenital anomaly or an acquired condition. Congenital hematotrachelos tends to present at puberty when menstrual blood is retained as a result of obstruction distal to the cervix such as from an imperforate hymen, complete transverse vaginal
OBSTETRICS & GYNECOLOGY
E
ndometrial adenocarcinoma is the most common gynecologic cancer in the United States, with more than 50,000 new cases and almost 8,600 deaths each year.1 Type I endometrioid adenocarcinoma represents approximately 80% of endometrial cancers and arises in the setting of continuous estrogen stimulation. Although common, the prognosis of endometrioid adenocarcinoma is generally favorable because approximately 75% of cases are diagnosed while confined to the uterine corpus. Factors associated From the Departments of Obstetrics and Gynecology and Pathology and Laboratory Medicine, University of Tennessee Health Science Center, and Department of Obstetrics and Gynecology, University of Tennessee Health Science Center—West Clinic, Memphis, Tennessee. Corresponding author: Michael A. Ulm, MD, MS, University of Tennessee Health Science Center, Department of Obstetrics and Gynecology, 853 Jefferson Avenue, Room E102, Memphis, TN 38163; e-mail: [email protected]. Financial Disclosure The authors did not report any potential conflicts of interest. © 2014 by The American College of Obstetricians and Gynecologists. Published by Lippincott Williams & Wilkins. ISSN: 0029-7844/14
VOL. 124, NO. 2, PART 2, AUGUST 2014
with clinical outcome include histologic grade, depth of myometrial invasion, lymphovascular space involvement, and lymph node involvement.2 Adenomyosis is present in 10% to 70% of hysterectomy specimens and is secondarily involved with endometrioid adenocarcinoma approximately 21% to 30% of the time when both conditions are present.3 Including our patient, 36 cases of extrauterine adenomyomas have been reported, and they are typically found in the adnexa.4 Endometrioid adenocarcinoma in an extrauterine adenomyoma found in our patient presents an interesting challenge in staging and management of extrauterine disease not attributed to metastasis from the uterine corpus. Our discussion encompasses the etiology of the lesion as well as current literature and recommendations regarding staging of endometrioid adenocarcinoma involving an extrauterine adenomyoma.
CASE A 49-year-old woman, gravida 0, with a 10-year history of metomenorrhagia presented to her gynecologist after being hospitalized for vaginal bleeding and significant anemia requiring blood transfusion. Endometrial biopsy was performed, with findings of a grade 2 endometrioid adenocarcinoma, and she was referred to a gynecologic oncologist. The patient’s medical history was significant for morbid obesity, chronic anemia, and hypertension. Her hypertension was controlled with multiple antihypertensive medications. She had undergone dilation and curettage for menorrhagia with benign pathology in 1983. She experienced menarche at age 9 years and had a history of regular menses until the past 10 years. She had not previously undergone evaluation for her metomenorrhagia and she had not undergone gynecologic evaluation in more than 15 years. She denied any history of contraceptive use or ever having sexual intercourse. On presentation, her vital signs were within normal limits (weight 240.4 lb, height 63 inches, and body mass index 42.6). She had a visually normal cervix and vagina with minimal bleeding. On examination, her uterus was estimated to be the size of that of a woman at 16 weeks of gestation, and it was compromised by a morbidly obese abdomen. A 3-cm firm mass suspicious for inguinal adenopathy was palpated in her left inguinal region. On questioning, the patient stated the inguinal mass had been palpable for an unknown number of years. Total abdominal hysterectomy, bilateral salpingo-oophorectomy, periaortic and pelvic lymph node dissection, and left inguinal lymph node dissection were surgically staged. During the left inguinal lymph node dissection, it was clear the mass was not arising from the lymph nodes but rather from the left round ligament, and it extended under the inguinal ligament into the left femoral triangle. Intraoperatively, gross section of the left inguinal mass was suspicious for metastatic disease. The right round ligament was grossly
Ulm et al
Carcinoma in an Extrauterine Adenomyoma
445
Fig. 1. Pathologic section from the extrauterine adenomyoma with foci of endometrioid adenocarcinoma surrounded by endometrial stroma and myometrium. Ulm. Carcinoma in an Extrauterine Adenomyoma. Obstet Gynecol 2014.
normal, and suture was ligated in the standard manner. The patient had an uncomplicated hospital course. Final pathology was International Federation of Gynecology and Obstetrics (FIGO) stage 1A, grade 2. The intrauterine mass was 11.5 cm at its greatest dimension, with 1.7 cm myometrial invasion or 45% of the myometrium, and without lymphatic or vascular space invasion. The uterine corpus had focal adenomyosis without endometrioid adenocarcinoma involvement. The fallopian tubes, ovaries, cervix, and lymph nodes were negative for malignancy. The pathology report of the tissue submitted as “left inguinal lymph node” contained a 3.8-cm
prominent smooth muscle nodule containing many endometrial glands demonstrating foci of adenocarcinoma. The glandular proliferation was contained within the smooth muscle, without infiltration into the soft tissue surrounding the smooth muscle (Fig. 1). The smooth muscle nodule and endometrial stroma within it were positive for desmin and CD10 immunostains, respectively (Fig. 2). Immunohistochemistry was positive for estrogen and progesterone receptors in 90 to 100% of the tumor cells in both the uterus and extrauterine adenomyoma. The constellation of morphologic and immunohistochemical findings were consistent with an extrauterine adenomyoma, focally involved with endometrioid adenocarcinoma, in the left round ligament. The findings were presented at Tumor Board, a multidisciplinary conference involving pathology, gynecologic oncology, radiation oncology, and interventional radiology. It was agreed that the extrauterine adenomyoma contained a second primary site of endometrioid adenocarcinoma and did not represent metastatic disease. The recommendation was made for follow-up every 3 months, with a computed tomography scan of the abdomen and pelvis every 6 months for the first 2 years. At her 6-month followup, she was doing well without evidence of disease.
COMMENT We performed a review of the literature using the MEDLINE-Ovid database. Our search was in the English language from 1950 to 2014; the search included the terms “extrauterine adenomyoma,” “uterus-like,” “adenomyoma cancer,” “adenomyoma carcinoma,” and “adenomyoma adenocarcinoma.” Including this patient, there are 36 cases of
Fig. 2. Pathologic section from the extrauterine adenomyoma with foci of endometrioid adnocarcinoma. A. Hematoxylin and eosin stain. B. Desmin stain for myometria stroma. C. CD10 stain for endometrial stroma. Both the desmin and CD10 stains show the glandular hyperplasia completely surrounded by myometrium and endometrial stroma, respectively. Ulm. Carcinoma in an Extrauterine Adenomyoma. Obstet Gynecol 2014.
446
Ulm et al
Carcinoma in an Extrauterine Adenomyoma
OBSTETRICS & GYNECOLOGY
extrauterine adenomyomas, or uterus-like masses, in the English literature (Table 1). Including our case, only two malignant uterus-like masses or extrauterine adenomyomas have been reported in the literature. Other reports have detailed masses ranging from 1 to 20 cm in size, with cystic components, adenomyosis, or large cavities lined with endometrial tissue.4–19 However, this is the first report of concurrent involvement of endometrioid adenocarcinoma in both an extrauterine adenomyoma and uterine fundus. Adenomyosis is defined as the intramyometrial presence of endometrial mucosa surrounded by reactive, hypertrophic myometrium. The etiology of adenomyosis is unknown. Endometrium and adjacent myometrium have a common embryological origin from the Mullerian ducts. The most often quoted hypothesis for the origin of adenomyosis is invagination of endometrial tissue between the smooth muscle fibers of the myometrium. This case, however, lends credence to the theory of adenomysosis formation from metaplasia of de novo ectopic endometrial tissue during embryologic development.20 During Mullerian duct differentiation, the female gubernaculum is formed from the caudal fold of the Wolffian body, which is provoked by the mesonephros elevating the covering peritoneum. It extends from the gonadal ridge to the future inguinal region and is composed of mesenchymal cells and muscular and extracellular matrix precursors. Although the origin of the female gubernaculum is controversial, it is generally accepted that the cranial portion forms the utero-ovarian ligament and the caudal portion forms the round ligament. Tumors in the inguinal canal involving the round ligament are rare and are typically leiomomyomas. Most published cases are associated with Mullerian agenesis and Rokitansky syndrome, forming indirect hernias.21 Although this patient did not have renal malformation, it is plausible to suggest that a portion of her Mullerian duct migrated along the urogenital ridge to form the extrauterine adenomyoma during embryologic development of the genital tract. Unopposed estrogen, in the setting of chronic anovulation, produced in adipose tissue has a mitogenic effect on endometrial cells.22 The majority of cases of endometrioid adenocarcinoma result from estrogenic stimulation of endometrial hyperplasia or endometrial intraepithelial neoplasia as a precursor lesion.2 We contend that unopposed estrogen is the cause of the uterine endometrioid adenocarcinoma and the endometrioid adenocarcinoma contained within the existing extrauterine adenomyoma.
VOL. 124, NO. 2, PART 2, AUGUST 2014
Table 1. Locations and Frequency of Sites of the 36 Extrauterine Adenomyomas Reported in the Literature Site
n
%
Ovary Broad ligament Abdomino-pelvic Pelvic wall Small bowel mesentary Conus medullaris Ovarian ligament Colon Inguinal canal Ileum Round ligament Obturator lymph node Posterior cul-de-sac Appendix
10 5 4 3 2 2 2 2 1 1 1 1 1 1
27.7 13.8 11.1 8.3 5.6 5.6 5.6 5.6 2.8 2.8 2.8 2.8 2.8 2.8
Data from references 4–19.
Hanley et al3 performed a retrospective analysis of 82 hysterectomy specimens with adenomyosis involved with endometrioid adenocarcinoma invading less than 50% of the myometrium (FIGO stage 1A). In otherwise low-stage tumors, they concluded that endometrioid adenocarcinoma involved in deeply located adenomyosis does not affect prognosis. They further state “myometrial-based foci of well-differentiated endometrioid adenocarcinoma, completely or partially surrounded by endometrial stroma, most likely represents tumor colonized adenomyosis.”3 According to the FIGO staging system for endometrial cancers, all metastatic disease located outside of the peritoneal cavity is stage IVB.2 In this case, the lesion was well-differentiated, completely surrounded by endometrial stroma, and completely resected from the inguinal canal. The extrauterine adenomyoma with focal endometrioid adenocarcinoma is a second primary site of endometrioid adenocarcinoma and not metastatic disease outside of the peritoneal cavity. The findings support the final diagnosis of FIGO stage 1A, grade 2 endometrioid adenocarcinoma. REFERENCES 1. Siegal R, Ma J, Zou Z, Jemal A. Cancer statistics, 2014. CA Cancer J Clin 2014;64:9–29. 2. Sorosky J. Endometrial cancer. Obstet Gynecol 2012;120: 383–97. 3. Hanley K, Dustin M, Stoler M, Atkins K. The significance of tumor involved adenomyosis in otherwise low-stage endometrioid adenocarcinoma. Int J Gynecol Pathol 2010;29:445–51. 4. Carinelli S, Motta F, Frontino G, Restelli E, Fedele L. Multiple extrauterine adenomyomas and uterine-like masses: case reports and review of the literature. Fertil Steril 2009;91:1956.e9–11.
Ulm et al
Carcinoma in an Extrauterine Adenomyoma
447
5. Rahilly MA, al-Nafussi A. Uterus-like mass if the ovary associated with endometrioid carcinoma. Histopathology 1991;18: 549–51. 6. Bulut AS, Sipahi TU. Abscessed uterine and extrauterine adenomyomas with uterus-like features in a 56-year-old woman. Case Rep Obstet Gynecol 2013;2013:238156. 7. Moghadamfalahi M, Metzinger DS. Multiple extrauterine adenomyomas presenting in upper abdomen and pelvis: a case report and brief review of the literature. Case Rep Obstet Gynecol 2013;2102:565901. 8. Sisodia SM, Khan WA, Goel A. Ovarian ligament adenomyoma: report of a rare entity with review of the literature. J Obstet Gynaecol Res 2012;38:724–8. 9. Khurana A, Mehta A, Sardana M. Extrauterine adenomyoma with uterus like features: a rare entity presenting 17 years posthysterectomy. Indian J Pathol Microbiol 2011;54:572–3. 10. Stewart CJ, Leung YC, Mathew R, McCartney AL. Extrauterine adenomyoma with atypical (Symplastic) smooth muscle cells: a report of 2 cases. Int J Gynecol Pathol 2009;28:23–8. 11. Kim JO, Baek JM, Jeung C, Park EK, Lee HN, Lee YS. A case of primary ovarian adenomyoma mimicking ovarian malignancy. Eur J Gynaecol Oncol 2011;32:103–6. 12. Bayar U, Demirtas E, Usubutun A, Basaran M, Esinler I, Yarali H. Ovarian adenomyoma following gonadotropin treatment for infertility. Reprod Biomed Online 2006;13:676–9. 13. Nechi S, Znaidi N, Rammah S, M’farej MK, Zermani R. Uterus-like mass of the broad ligament. Int J Gynaecol Obstet 2013;123:249–50. 14. Sharma MC, Sarkar C, Jain D, Suri V, Garg A, Vaishya S. Uterus-like mass of mullerian origin in the lumbosacral region
Endometrial Adenocarcinoma Presenting as a Hematotrachelos Christopher M. Sauer, MD, MBA, Sudeshna Chatterjee, MD, Gary M. Israel, MD, and Peter E. Schwartz, MD BACKGROUND: Hematotrachelos, distension of the uterine cervix with accumulated blood, is an extremely rare condition resulting from a congenital anomaly or an acquired condition. We present a case in which an
causing cord tethering. Report of two cases. J Neurosurg Spine 2007;6:73–6. 15. Seki A, Maeshima A, Nakaqawa H, Shiraishi J, Murata Y, Arai H, et al. A subserosal uterus-like mass presenting after a sliding hernia of the ovary and endometriosis: a rare entity with a discussion of histogenesis. Fertil Steril 2011;95:1788.e15–19. 16. Shin SY, Kim HJ, Kim YW, Lee KY. CT characteristics of a uterus-like mass in the sigmoid mesocolon. Br J Radiol 2011;84:e1–3. 17. Carvalho FM, Carvalho JP, Pereira RM, Ceccato BP Jr, Lacordia R, Baracat EC. Leiomyomatosis peritonealis disseminata associated with endometriosis and multiple uterus-like masses: report of two cases. Clin Med Insights 2012;5:63–8. 18. Liang YJ, Hao Q, Wu YZ, Wu B. Uterus-like mass in the left broad ligament misdiagnosed as a malformation of the uterus: a case report of a rare condition and review of the literature. Fertil Steril 2010;93:1347.e13–6. 19. Na K, Kim GY, Won KY, Kim HS, Kim SW, Lee CH, et al. Extrapelvic uterus-like masses presenting as colonic submucosal tumor: a case study and review of literature. Korean J Pathol 2013;47:177–81. 20. Acien P, Sanchez del Campo F, Mayol MJ, Acien M. The female gubernaculum: role in the embryology and development of the genital tract and in the possible genesis of malformations. Eur J Obstet Gynecol Reprod Biol 2011;159:426–32. 21. Ferenczy A. Pathophysiology of adenomyosis. Hum Reprod Update 1998;4:312–22. 22. Lindemann K, Vatten LJ, Ellstrom-Engh M, Eskild A. Body mass, diabetes and smoking, and endometrial cancer risk: a follow-up study. Br J Cancer 2008;98:1582–5.
acquired hematotrachelos was the presenting sign of endometrial cancer. CASE: An asymptomatic 66-year-old woman was found to have a bulging cervix during a well-woman visit. Further workup revealed a hematotrachelos and an underlying endometrial adenocarcinoma. She was treated with surgery and adjuvant radiotherapy. CONCLUSION: A hematotrachelos, although rare, can prevent vaginal bleeding, which is often the earliest symptom of a uterine malignancy. This case report illustrates the potential importance of the pelvic examination as part of the well-woman physical examination, because it led to the discovery of early-stage endometrial cancer. (Obstet Gynecol 2014;124:448–51)
From the Departments of Obstetrics, Gynecology, and Reproductive Sciences and Diagnostic Radiology, Yale University School of Medicine, New Haven, Connecticut. Corresponding author: Christopher M. Sauer, BA, 123 York Street, Apt 12A, New Haven, CT 06511; e-mail: [email protected]. Financial Disclosure The authors did not report any potential conflicts of interest. © 2014 by The American College of Obstetricians and Gynecologists. Published by Lippincott Williams & Wilkins. ISSN: 0029-7844/14
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Sauer et al
Hematotrachelos
DOI: 10.1097/AOG.0000000000000281
H
ematotrachelos is a rare condition in which the uterine cervix becomes distended with blood, resulting from a congenital anomaly or an acquired condition. Congenital hematotrachelos tends to present at puberty when menstrual blood is retained as a result of obstruction distal to the cervix such as from an imperforate hymen, complete transverse vaginal
OBSTETRICS & GYNECOLOGY
