Granulosa cell tumors constitute less than 5 % of all ovarian tumors. Unlike epithelial ovarian tumors, they occur in a younger age group, are usually detected in an early stage and often have features of hyperestrogenism. The presenting symptoms are usually nonspecific with abdominal pain or distension. They follow an indolent course and are characterized by a long natural history. Mutation of FOXL2 (402C->G) seen in 97 % of adult GCT may be pathognomonic for adult GCT. Only stage of the disease has been consistently shown in various studies to affect survival of patients with GCT. The initial management of patients, for whom fertility is not an issue, is total abdominal hysterectomy, bilateral salpingo-oophorectomy and removal of all gross disease. Nodal dissection is not a significant factor for survival and is not recommended in surgical staging of GCT. Fertility preserving surgery with unilateral salpingooophorectomy is feasible in young patients with stage Ia GCT. Patients with early stage disease (stage I and II) have a very good prognosis with 5 year DFS and OS of 89 % and 99 % respectively and these groups of patients usually don't require any postoperative treatment. Patients with stage Ic disease associated with poor prognostic factors like large tumor size or high mitotic index and stage II, have a higher chance of relapse, and may benefit with postoperative treatment but role of chemotherapy is still debatable. In advanced stage disease (stage III and IV) the 5 year DFS and OS disease was 72 % and 80 % respectively hence the option of postoperative treatment with 6 cycles of BEP should be considered in this group. Recently paclitaxel is being investigated as an effective tool in GCT. The efficacy of radiation in GCT is not well defined but in optimally debulked cases postoperative radiation is a viable option. Due to high chance of recurrence even years after apparent clinical cure of the primary tumor, lifelong follow up with clinical examination and tumor markers like inhibin B is recommended. About 25 % GCT develop recurrence and the median time to recur is usually 4-5 years. Most recurrences are intraperitoneal and usually a complete debulking of the disease is feasible even in the recurrent setting. Postoperative chemotherapy (platinum based) is usually given after surgery more so in cases with widespread disease or after suboptimal cytoreduction. Recurrent chemoresistant, progressive non-responding GCT or patients with high surgical risk are ideal candidates for targeted therapy.

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Sister Joseph nodule is a metastatic umbilical lesion secondary to a primary malignancy of any viscera. It can be a presenting symptom (a sign of undiagnosed malignancy) or a symptom or sign of progression or recurrence in a known case. Its incidence is 1–3% of all intra-abdominal or pelvic malignancies. Here, we present 4 such cases, with Sister Joseph nodule as a finding of (1) presentation in a case of gallbladder carcinoma, (2) progression in a case of malignant gastrointestinal stromal tumour, (3) recurrence in a case of ovarian carcinoma, and (4) presentation in a case of rectal carcinoma. The clinicopathologic features of all 4 patients are discussed, and the related literature is briefly reviewed.

Objective:The objective of this study is to evaluate the pattern of care and survival outcome in patients with malignant ovarian germ cell tumors (MOGCTs).Materials and Methods:Between January 2004 and August 2017, 50 patients with MOGCT were identified at Amrita Institute of Medical Sciences and 48 included in analyses. Histologic subtypes were as follows: dysgerminoma 11; immature teratoma 16; yolk sac tumor 3; and mixed germ cell tumor 18. 31 (64.6% patients belonged to Stage I and 17 (35.4%) patients were advanced stage (Stage II-IV).Results:Median follow-up period was 34 months (range: 1–241 months). The 5- and 10-year disease-free survival (DFS) and overall survival (OS) for the entire cohort were 87.5% and 94.4%, respectively. DFS and OS of incomplete surgery Stage I patients 28.6% and 68.6%, respectively, were significantly lower than completely staged patients 100%. Out of 8 incomplete surgery patients, 5 recurred of which 2 died of disease within 4 and 9 months of recurrence. There was no survival difference with comprehensive surgical staging (CSS) and pediatric surgical staging (PSS) in Stage I MOGCT (DFS and OS 100%). Stage I dysgerminoma kept on active surveillance after PSS had equivalent survival of 100%. There was no survival difference in advanced stage MOGCT treated with primary debulking surgery and neoadjuvant chemotherapy (NAC) followed by fertility-sparing surgery (DFS and OS 100%).Conclusion:Incomplete surgery in Stage I MOGCT was associated with poor survival. There was no survival difference with CSS and PSS. NAC followed by surgery could be a reasonable option for patients of advanced stage MOGCT.

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This is the first and largest genetic map the DPYD variants associated with adverse drug reaction to 5-FU in south Asian population. Our study highlights ethnic differences in allelic frequencies.

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