Unusual presentation of more common disease/injury
CASE REPORT
Sister Mary Joseph’s nodule as the first and only sign of recurrence in a case of stage Ia carcinoma ovary Prerna Gupta, Aruna Kumari, Alka Kriplani, Neerja Bhatla Department of Obstetrics and Gynecology, AIIMS, New Delhi, India Correspondence to Dr Prerna Gupta, [email protected]
SUMMARY Sister Mary Joseph’s nodule, a term coined by Sir Hamilton Bailey, describes the umbilical nodule associated with advanced intra-abdominal malignancy. It signifies extensive intra-abdominal disease with a poor prognosis and a median survival of 4–14 months. We describe a case in which discharge from umbilical nodule was the first sign of recurrence in carcinoma ovary. On laparotomy, besides extra-abdominal umbilical deposit there was no peritoneal disease.
BACKGROUND Sister Mary Joseph’s nodule, a term coined by Sir Hamilton Bailey, describes the umbilical nodule associated with advanced intra-abdominal malignancy.1 It signifies extensive intra-abdominal disease with a poor prognosis and a median survival of 4–14 months.1 We describe a case in which discharge from the umbilical nodule was the first sign of recurrence in carcinoma ovary. On laparotomy, besides extra-abdominal umbilical deposit there was no peritoneal disease.
CASE PRESENTATION A 56-year-old postmenopausal woman presented to our outpatient department with symptoms of serous discharge from the umbilicus for 3 months. She had a history of total abdominal hysterectomy with bilateral salpingo-oopherectomy and infracolic omentectomy performed 2 years ago for stage Ia ovarian cancer. The histopathology showed an enlarged left ovary (15×16 cm), multiloculated with papillary excrescences and intact capsule. The uterus, right ovary and omentum were unremarkable. On microscopy, the left ovary showed serous papillary adenocarcinoma, with no tumour elsewhere in the specimen. Based on this pathology, stage Ia carcinoma ovary was diagnosed. No chemotherapy was advised and the patient was on regular follow-up. Multiple granulations were found in the umbilicus with no other palpable mass felt on the abdominal examination. Pelvic examination revealed a healthy vault and no nodularity in pouch of Douglas.
To cite: Gupta P, Kumari A, Kriplani A, et al. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2013201779
at umbilical level. No obvious intra-abdominal extension was seen. A fine-needle aspiration cytology performed on the granulomatous umbilical mass showed metastatic papillary adenocarcinoma.
TREATMENT In view of recurrence, the patient was planned for exploratory laparotomy and wide local excision of umbilical mass. A midline incision was performed to proceed with laparotomy. Intraoperative peritoneal cytology showed a 2 cm×2 cm subcutaneous umbilical nodule with noextension intra-abdominally. A wide local excision performed for the umbilical mass (figure 1). Interestingly, no signs of peritoneal disease were found, hence random peritoneal biopsies and lymph node sampling were performed.
OUTCOME AND FOLLOW-UP Histopathology revealed metastatic papillary adenocarcinoma in umbilical mass with free margins. There were no tumour deposits in peritoneal biopsies and lymph node samples. The patient was discharged in stable condition from hospital and planned for chemotherapy.
DISCUSSION Sister Mary Joseph nodule is a rare clinical manifestation of advanced intra-abdominal malignancies mainly gastrointestinal and ovarian tumours. However, in 10–15% of cases the primary site remains elusive.2 This sign can be the first sign of disease or an indicator of recurrence. Previously published reports have shown it to be a marker of extensive disease.3 4 However, in our case, it was
INVESTIGATIONS In blood investigations, the patient’s haemogram, liver and renal function tests were within normal limits. Serum CA-125 was normal at 14.6 IU/L. A contrast-enhanced CT scan whole abdomen performed showed a 2.5×2.5 cm subcutaneous nodule
Gupta P, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2013-201779
Figure 1 Gross photograph of umbilical nodule after wide local excision. 1
Unusual presentation of more common disease/injury novel to see no sign of intra-abdominal disease macroscopically as well as on microscopy of peritoneal biopsies.
Competing interests None. Patient consent Obtained. Provenance and peer review Not commissioned; externally peer reviewed.
Learning points ▸ Sister Mary Joseph nodule is a manifestation of intra-abdominal malignancy. ▸ It is commonly associated with gastrointestinal and gynaecological malignancies. ▸ Surgery with wide local excision followed by chemotherapy is helpful in patients with only localised umbilical metastasis. In patients with wide peritoneal disease, surgery has a limited role.
REFERENCES 1
2
3
4
Al-Marshat F, Sibiany AM. Sister Mary Joseph’s nodule of the umbilicus: is it always of gastric origin? A review of eight cases at different sites of origin. Indian J Cancer 2010;47:65–9. Falchi M, Cecchini G, Derchi LE. Umbilical metastasis as first sign of cecal carcinoma in a cirrhotic patient (Sister Mary Joseph nodule). Report of a case. Radiol Med 1999;98:94–6. Chalya PL, Mabula JB, Rambau PF, et al. Sister Mary Joseph’s nodule at a University teaching hospital in northwestern Tanzania: a retrospective review of 34 cases. World J Surg Oncol 2013;11:151. Nikolaou M, Zyli P, Stamenkovic S, et al. Sister Mary Joseph’s nodule in advanced ovarian cancer. J Obstet Gynaecol 2013;33:214–15.
Copyright 2014 BMJ Publishing Group. All rights reserved. For permission to reuse any of this content visit http://group.bmj.com/group/rights-licensing/permissions. BMJ Case Report Fellows may re-use this article for personal use and teaching without any further permission. Become a Fellow of BMJ Case Reports today and you can: ▸ Submit as many cases as you like ▸ Enjoy fast sympathetic peer review and rapid publication of accepted articles ▸ Access all the published articles ▸ Re-use any of the published material for personal use and teaching without further permission For information on Institutional Fellowships contact [email protected] Visit casereports.bmj.com for more articles like this and to become a Fellow
2
Gupta P, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2013-201779
CASE REPORT
Sister Mary Joseph’s nodule as the first and only sign of recurrence in a case of stage Ia carcinoma ovary Prerna Gupta, Aruna Kumari, Alka Kriplani, Neerja Bhatla Department of Obstetrics and Gynecology, AIIMS, New Delhi, India Correspondence to Dr Prerna Gupta, [email protected]
SUMMARY Sister Mary Joseph’s nodule, a term coined by Sir Hamilton Bailey, describes the umbilical nodule associated with advanced intra-abdominal malignancy. It signifies extensive intra-abdominal disease with a poor prognosis and a median survival of 4–14 months. We describe a case in which discharge from umbilical nodule was the first sign of recurrence in carcinoma ovary. On laparotomy, besides extra-abdominal umbilical deposit there was no peritoneal disease.
BACKGROUND Sister Mary Joseph’s nodule, a term coined by Sir Hamilton Bailey, describes the umbilical nodule associated with advanced intra-abdominal malignancy.1 It signifies extensive intra-abdominal disease with a poor prognosis and a median survival of 4–14 months.1 We describe a case in which discharge from the umbilical nodule was the first sign of recurrence in carcinoma ovary. On laparotomy, besides extra-abdominal umbilical deposit there was no peritoneal disease.
CASE PRESENTATION A 56-year-old postmenopausal woman presented to our outpatient department with symptoms of serous discharge from the umbilicus for 3 months. She had a history of total abdominal hysterectomy with bilateral salpingo-oopherectomy and infracolic omentectomy performed 2 years ago for stage Ia ovarian cancer. The histopathology showed an enlarged left ovary (15×16 cm), multiloculated with papillary excrescences and intact capsule. The uterus, right ovary and omentum were unremarkable. On microscopy, the left ovary showed serous papillary adenocarcinoma, with no tumour elsewhere in the specimen. Based on this pathology, stage Ia carcinoma ovary was diagnosed. No chemotherapy was advised and the patient was on regular follow-up. Multiple granulations were found in the umbilicus with no other palpable mass felt on the abdominal examination. Pelvic examination revealed a healthy vault and no nodularity in pouch of Douglas.
To cite: Gupta P, Kumari A, Kriplani A, et al. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2013201779
at umbilical level. No obvious intra-abdominal extension was seen. A fine-needle aspiration cytology performed on the granulomatous umbilical mass showed metastatic papillary adenocarcinoma.
TREATMENT In view of recurrence, the patient was planned for exploratory laparotomy and wide local excision of umbilical mass. A midline incision was performed to proceed with laparotomy. Intraoperative peritoneal cytology showed a 2 cm×2 cm subcutaneous umbilical nodule with noextension intra-abdominally. A wide local excision performed for the umbilical mass (figure 1). Interestingly, no signs of peritoneal disease were found, hence random peritoneal biopsies and lymph node sampling were performed.
OUTCOME AND FOLLOW-UP Histopathology revealed metastatic papillary adenocarcinoma in umbilical mass with free margins. There were no tumour deposits in peritoneal biopsies and lymph node samples. The patient was discharged in stable condition from hospital and planned for chemotherapy.
DISCUSSION Sister Mary Joseph nodule is a rare clinical manifestation of advanced intra-abdominal malignancies mainly gastrointestinal and ovarian tumours. However, in 10–15% of cases the primary site remains elusive.2 This sign can be the first sign of disease or an indicator of recurrence. Previously published reports have shown it to be a marker of extensive disease.3 4 However, in our case, it was
INVESTIGATIONS In blood investigations, the patient’s haemogram, liver and renal function tests were within normal limits. Serum CA-125 was normal at 14.6 IU/L. A contrast-enhanced CT scan whole abdomen performed showed a 2.5×2.5 cm subcutaneous nodule
Gupta P, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2013-201779
Figure 1 Gross photograph of umbilical nodule after wide local excision. 1
Unusual presentation of more common disease/injury novel to see no sign of intra-abdominal disease macroscopically as well as on microscopy of peritoneal biopsies.
Competing interests None. Patient consent Obtained. Provenance and peer review Not commissioned; externally peer reviewed.
Learning points ▸ Sister Mary Joseph nodule is a manifestation of intra-abdominal malignancy. ▸ It is commonly associated with gastrointestinal and gynaecological malignancies. ▸ Surgery with wide local excision followed by chemotherapy is helpful in patients with only localised umbilical metastasis. In patients with wide peritoneal disease, surgery has a limited role.
REFERENCES 1
2
3
4
Al-Marshat F, Sibiany AM. Sister Mary Joseph’s nodule of the umbilicus: is it always of gastric origin? A review of eight cases at different sites of origin. Indian J Cancer 2010;47:65–9. Falchi M, Cecchini G, Derchi LE. Umbilical metastasis as first sign of cecal carcinoma in a cirrhotic patient (Sister Mary Joseph nodule). Report of a case. Radiol Med 1999;98:94–6. Chalya PL, Mabula JB, Rambau PF, et al. Sister Mary Joseph’s nodule at a University teaching hospital in northwestern Tanzania: a retrospective review of 34 cases. World J Surg Oncol 2013;11:151. Nikolaou M, Zyli P, Stamenkovic S, et al. Sister Mary Joseph’s nodule in advanced ovarian cancer. J Obstet Gynaecol 2013;33:214–15.
Copyright 2014 BMJ Publishing Group. All rights reserved. For permission to reuse any of this content visit http://group.bmj.com/group/rights-licensing/permissions. BMJ Case Report Fellows may re-use this article for personal use and teaching without any further permission. Become a Fellow of BMJ Case Reports today and you can: ▸ Submit as many cases as you like ▸ Enjoy fast sympathetic peer review and rapid publication of accepted articles ▸ Access all the published articles ▸ Re-use any of the published material for personal use and teaching without further permission For information on Institutional Fellowships contact [email protected] Visit casereports.bmj.com for more articles like this and to become a Fellow
2
Gupta P, et al. BMJ Case Rep 2014. doi:10.1136/bcr-2013-201779
