Gynecologic Oncology Reports 2 (2012) 110–111
Contents lists available at SciVerse ScienceDirect
Gynecologic Oncology Reports journal homepage: www.elsevier.com/locate/gynor
Case report
Endometrial cancer diagnosed by Sister Mary Joseph nodule biopsy: Case report Christy Nolan a,⁎, Diane Semer b a b
Brody School of Medicine at East Carolina University, PCMH TA 165, Greenville, NC 27834, USA Gynecologic Oncologist, Physicians East, P.A., USA
a r t i c l e
i n f o
Article history: Received 3 April 2012 Accepted 11 June 2012 Available online 17 June 2012 Keywords: Sister Mary Joseph nodule Papillary serous endometrial cancer Umbilical metastasis
Introduction A Sister Mary Joseph nodule is an umbilical metastasis found in less than 3% of patients with intra-abdominal or pelvic malignancy (Piura et al., 2006). These rare nodules have a wide range of presentations but most typically are noted to be a painful, ulcerated mass with serosanguineous to bloody discharge (Levine et al., 2010). Although rarely reported, the presence of a Sister Mary Joseph nodule can be an important diagnostic and prognostic factor in the assessment of gynecologic oncology patients. In this case we describe a patient who presented with a likely gynecologic malignancy and notable Sister Mary Joseph nodule. As an endometrial or cervical biopsy was difficult due to heavy vaginal bleeding, biopsy of the umbilical nodule allowed for an early tissue diagnosis of papillary serous adenocarcinoma and subsequently helped to guide staging and treatment options. Case report A 61 year old African American female presented to the hospital with complaints of abdominal pain, enlarging umbilical mass, and nausea with vomiting. She stated that she began to feel poorly over the month prior to presentation with multiple nonspecific complaints including an enlarging umbilical mass. She stated that despite being 61, she had never experienced menopause and continued to have ⁎ Corresponding author. Fax: + 1 252 744 3824. E-mail address: [email protected] (C. Nolan). 2211-338X/$ – see front matter © 2012 Elsevier Inc. All rights reserved. doi:10.1016/j.gynor.2012.06.003
regular menses without interruption. She did note that she had not received any health care within the past several years. Her physical exam was concerning for abdominal distention and a 2 × 2 cm friable, beefy red lesion within the umbilicus draining a serosanguineous fluid. On pelvic exam, she was noted to have a stenotic and matted cervix without obvious lesion but exam was limited by heavy vaginal bleeding. An endometrial biopsy could not be performed due to the inability to pass a curette beyond the noted cervical stenosis. At this time attention was turned to her umbilical lesion, which was easily biopsied. Pathology of her umbilical mass returned poorly differentiated metastatic papillary carcinoma. A CT Scan was performed on admission showing a partial small bowel obstruction, thickened endometrium, peritoneal carcinomatosis, umbilical hernia, left ovarian dermoid and possible metastatic disease to the lower lung fields. The patient was given a preliminary diagnosis of metastatic papillary serous endometrial carcinoma. Due to ongoing heavy vaginal bleeding and persistent partial small bowel obstruction the patient was taken for an exploratory laparotomy. Surgical exploration was notable for a small bowel obstruction secondary to tumor entrapment. The tumor had replaced her omentum and was found throughout her abdomen, including an exophytic growth of tumor into and through her umbilicus. The uterus was noted to be enlarged with the tumor extending through the myometrium and obliterating the vesicovaginal space. Maximal possible cytoreductive surgery was undertaken with a supercervical hysterectomy, bilateral salpingo-oophorectomy, omentectomy, small bowel resection and generalized debulking with umbilical resection. Total hysterectomy was not possible due to the large amount of tumor invading the cervicovesical plane and endocervical areas. Final pathology was consistent with the endometrial papillary serous adenocarcinoma. The patient's postoperative course was unremarkable and she was discharged home within a few days of surgery. She received four doses of chemotherapy with carboplatin and paclitaxel, but continued to have rapid progression of disease. She ultimately died from her disease less than four months from the primary diagnosis. Discussion The term “Sister Mary Joseph Nodule” was first coined by Dr. Hamilton Bailey in his textbook, Physical Signs of Clinical Surgery (Powell, 2011), as a reference to Sister Mary Joseph's incidental observation of umbilical nodules in patients with advanced cancer. There are multiple hypothesized modes of metastasis to the umbilicus including lymphatic drainage from the para-aortic nodes, direct
C. Nolan, D. Semer / Gynecologic Oncology Reports 2 (2012) 110–111
extension from the peritoneum and extension of tumor along embryonic remnants (Powell, 2011). Metastatic cancer to the umbilicus is a rare occurrence with more than half of the cases originating from a gastrointestinal cancer and a smaller portion found to arise from gynecologic or unknown origins. Of the known cases of Sister Mary Joseph nodule related to gynecologic cancers (28% of all known cases) approximately 34% are ovarian, 12% are uterine and only 5% are cervical in origin (Levine et al., 2010). Endometrial cancer remains the most common female genital tract malignancy with an estimated 46,000 cases diagnosed in 2011 alone. Papillary serous histology is an infrequent but aggressive form of cancer that accounts for approximately 10% of all endometrial cancer. This diagnosis carries an overall poor prognosis with significant risk for extrauterine disease regardless of the depth of myometrial invasion. Uterine papillary serous carcinoma has a recurrence pattern similar to epithelial ovarian cancer and is staged accordingly. These patients should be referred to a gynecologic oncologist whenever possible for surgical resection and optimal treatment. The presentation of a Sister Mary Joseph nodule can be quite variable ranging from a hard and irregular nodule to a soft and painfully ulcerated mass (Levine et al., 2010). When present, this nodule is often the first sign of intra-abdominal or pelvic malignancy. This case serves to highlight the importance of a comprehensive physical exam in all patients, especially those with suspected malignancy. The Sister Mary Joseph nodule is one of the few external manifestations of underlying malignancy that can aid the surgeon in obtaining an early tissue diagnosis of malignancy in a difficult clinical picture. Quite often, the original pre-
111
sentation of advanced gynecological malignancies such as endometrial cancer and cervical cancer can overlap. In this case the patient's presentation was certainly concerning for overt endometrial cancer given her persistent post-menopausal bleeding and imaging; however an endophytic cervical cancer could not be ruled out given the firm and matted appearance of her cervix on pelvic exam. The preliminary pathology report obtained from her umbilical nodule helped to affirm the decision to have her undergo surgical staging and debulking for endometrial cancer, as opposed to primary pelvic radiation for cervical cancer. While the presence of a Sister Mary Joseph nodule by definition denotes advanced cancer, it should not preclude aggressive therapy for endometrial cancer. The majority of current studies suggest that these patients benefit from an aggressive approach including both surgery and chemotherapy (Majmudar et al., 1991). Conflict of interest statement The authors have no conflict of interest.
References Levine, D., Miller, S., Al-Dawsari, N., Barak, O., Gottlieb, A.B., 2010. Paraneoplastic dermatoses associated with gynecologic and breast malignancies. Obstet. Gynecol. Surv. 65 (7), 455–461. Majmudar, B., Wiskind, A.K., Croft, B.N., Dudley, A.G., 1991. The Sister (Mary) Joseph nodule: its significance in gynecology. Gynecol. Oncol. 40 (2), 152–159. Piura, B., Meirovitz, M., Bayme, M., Shaco-Levy, R., 2006. Sister Mary Joseph's nodule originating from endometrial carcinoma incidentally detected during surgery for an umbilical hernia: a case report. Arch. Gynecol. Obstet. 274 (6), 385–388. Powell, J.L., 2011. Powell's pearls: eponyms in medical and surgical history. Sister Joseph's nodule; Sister Mary Joseph (1856–1939). J. Surg. Educ. 68 (5), 442–443.
Contents lists available at SciVerse ScienceDirect
Gynecologic Oncology Reports journal homepage: www.elsevier.com/locate/gynor
Case report
Endometrial cancer diagnosed by Sister Mary Joseph nodule biopsy: Case report Christy Nolan a,⁎, Diane Semer b a b
Brody School of Medicine at East Carolina University, PCMH TA 165, Greenville, NC 27834, USA Gynecologic Oncologist, Physicians East, P.A., USA
a r t i c l e
i n f o
Article history: Received 3 April 2012 Accepted 11 June 2012 Available online 17 June 2012 Keywords: Sister Mary Joseph nodule Papillary serous endometrial cancer Umbilical metastasis
Introduction A Sister Mary Joseph nodule is an umbilical metastasis found in less than 3% of patients with intra-abdominal or pelvic malignancy (Piura et al., 2006). These rare nodules have a wide range of presentations but most typically are noted to be a painful, ulcerated mass with serosanguineous to bloody discharge (Levine et al., 2010). Although rarely reported, the presence of a Sister Mary Joseph nodule can be an important diagnostic and prognostic factor in the assessment of gynecologic oncology patients. In this case we describe a patient who presented with a likely gynecologic malignancy and notable Sister Mary Joseph nodule. As an endometrial or cervical biopsy was difficult due to heavy vaginal bleeding, biopsy of the umbilical nodule allowed for an early tissue diagnosis of papillary serous adenocarcinoma and subsequently helped to guide staging and treatment options. Case report A 61 year old African American female presented to the hospital with complaints of abdominal pain, enlarging umbilical mass, and nausea with vomiting. She stated that she began to feel poorly over the month prior to presentation with multiple nonspecific complaints including an enlarging umbilical mass. She stated that despite being 61, she had never experienced menopause and continued to have ⁎ Corresponding author. Fax: + 1 252 744 3824. E-mail address: [email protected] (C. Nolan). 2211-338X/$ – see front matter © 2012 Elsevier Inc. All rights reserved. doi:10.1016/j.gynor.2012.06.003
regular menses without interruption. She did note that she had not received any health care within the past several years. Her physical exam was concerning for abdominal distention and a 2 × 2 cm friable, beefy red lesion within the umbilicus draining a serosanguineous fluid. On pelvic exam, she was noted to have a stenotic and matted cervix without obvious lesion but exam was limited by heavy vaginal bleeding. An endometrial biopsy could not be performed due to the inability to pass a curette beyond the noted cervical stenosis. At this time attention was turned to her umbilical lesion, which was easily biopsied. Pathology of her umbilical mass returned poorly differentiated metastatic papillary carcinoma. A CT Scan was performed on admission showing a partial small bowel obstruction, thickened endometrium, peritoneal carcinomatosis, umbilical hernia, left ovarian dermoid and possible metastatic disease to the lower lung fields. The patient was given a preliminary diagnosis of metastatic papillary serous endometrial carcinoma. Due to ongoing heavy vaginal bleeding and persistent partial small bowel obstruction the patient was taken for an exploratory laparotomy. Surgical exploration was notable for a small bowel obstruction secondary to tumor entrapment. The tumor had replaced her omentum and was found throughout her abdomen, including an exophytic growth of tumor into and through her umbilicus. The uterus was noted to be enlarged with the tumor extending through the myometrium and obliterating the vesicovaginal space. Maximal possible cytoreductive surgery was undertaken with a supercervical hysterectomy, bilateral salpingo-oophorectomy, omentectomy, small bowel resection and generalized debulking with umbilical resection. Total hysterectomy was not possible due to the large amount of tumor invading the cervicovesical plane and endocervical areas. Final pathology was consistent with the endometrial papillary serous adenocarcinoma. The patient's postoperative course was unremarkable and she was discharged home within a few days of surgery. She received four doses of chemotherapy with carboplatin and paclitaxel, but continued to have rapid progression of disease. She ultimately died from her disease less than four months from the primary diagnosis. Discussion The term “Sister Mary Joseph Nodule” was first coined by Dr. Hamilton Bailey in his textbook, Physical Signs of Clinical Surgery (Powell, 2011), as a reference to Sister Mary Joseph's incidental observation of umbilical nodules in patients with advanced cancer. There are multiple hypothesized modes of metastasis to the umbilicus including lymphatic drainage from the para-aortic nodes, direct
C. Nolan, D. Semer / Gynecologic Oncology Reports 2 (2012) 110–111
extension from the peritoneum and extension of tumor along embryonic remnants (Powell, 2011). Metastatic cancer to the umbilicus is a rare occurrence with more than half of the cases originating from a gastrointestinal cancer and a smaller portion found to arise from gynecologic or unknown origins. Of the known cases of Sister Mary Joseph nodule related to gynecologic cancers (28% of all known cases) approximately 34% are ovarian, 12% are uterine and only 5% are cervical in origin (Levine et al., 2010). Endometrial cancer remains the most common female genital tract malignancy with an estimated 46,000 cases diagnosed in 2011 alone. Papillary serous histology is an infrequent but aggressive form of cancer that accounts for approximately 10% of all endometrial cancer. This diagnosis carries an overall poor prognosis with significant risk for extrauterine disease regardless of the depth of myometrial invasion. Uterine papillary serous carcinoma has a recurrence pattern similar to epithelial ovarian cancer and is staged accordingly. These patients should be referred to a gynecologic oncologist whenever possible for surgical resection and optimal treatment. The presentation of a Sister Mary Joseph nodule can be quite variable ranging from a hard and irregular nodule to a soft and painfully ulcerated mass (Levine et al., 2010). When present, this nodule is often the first sign of intra-abdominal or pelvic malignancy. This case serves to highlight the importance of a comprehensive physical exam in all patients, especially those with suspected malignancy. The Sister Mary Joseph nodule is one of the few external manifestations of underlying malignancy that can aid the surgeon in obtaining an early tissue diagnosis of malignancy in a difficult clinical picture. Quite often, the original pre-
111
sentation of advanced gynecological malignancies such as endometrial cancer and cervical cancer can overlap. In this case the patient's presentation was certainly concerning for overt endometrial cancer given her persistent post-menopausal bleeding and imaging; however an endophytic cervical cancer could not be ruled out given the firm and matted appearance of her cervix on pelvic exam. The preliminary pathology report obtained from her umbilical nodule helped to affirm the decision to have her undergo surgical staging and debulking for endometrial cancer, as opposed to primary pelvic radiation for cervical cancer. While the presence of a Sister Mary Joseph nodule by definition denotes advanced cancer, it should not preclude aggressive therapy for endometrial cancer. The majority of current studies suggest that these patients benefit from an aggressive approach including both surgery and chemotherapy (Majmudar et al., 1991). Conflict of interest statement The authors have no conflict of interest.
References Levine, D., Miller, S., Al-Dawsari, N., Barak, O., Gottlieb, A.B., 2010. Paraneoplastic dermatoses associated with gynecologic and breast malignancies. Obstet. Gynecol. Surv. 65 (7), 455–461. Majmudar, B., Wiskind, A.K., Croft, B.N., Dudley, A.G., 1991. The Sister (Mary) Joseph nodule: its significance in gynecology. Gynecol. Oncol. 40 (2), 152–159. Piura, B., Meirovitz, M., Bayme, M., Shaco-Levy, R., 2006. Sister Mary Joseph's nodule originating from endometrial carcinoma incidentally detected during surgery for an umbilical hernia: a case report. Arch. Gynecol. Obstet. 274 (6), 385–388. Powell, J.L., 2011. Powell's pearls: eponyms in medical and surgical history. Sister Joseph's nodule; Sister Mary Joseph (1856–1939). J. Surg. Educ. 68 (5), 442–443.
