YO4 - pelvic nut carcinoma

Case summary

DIAGNOSIS: Carcinoma of unknown primary with adnexal mass and hilar nodal mass BREIF HISTORY : 21/F, Student, Oligomenorrhea, (menses every 5-6 wks) LMP: 26/1/19 Developed on and off fever from feb 2019 associated with chest pain and cough a.w Rt Lumbar region pain CECT (25/2/19) : Right hilar and subcarinal L.N. mass 2.9 X 3.6 cm Right Upper Pulmonary vein compression Complete obstruction of middle lobe bronchus with collapse consolidation. Mild Right sided Pleural effusion .Sub Carinal Lymph Node Bx was done and evaluated further. Patient received antibiotics for few weeks and ATT was started and given for few days Negative for TB and other infection. HPR : Suspicious of thymic tumour IHC : CK (NNF) , ENA, p63 : Strongly positive TTF, CD20, CD3, CD68, CD 30 _ve CD 117, OcT 4, PAX8, CD 5 : Negative. IHC Positive for NUT Antibody . USG A+P (15/3/19) : Rt adnexal mass 45 X 40 mm Lt ovary . PET CECT (16/3/19) Rt Hilar necrotic mass 5.9 X 8.0 X 5.9 cm SUV 11.4 Multiple FDG Rt paratracheal L.N. Rt adnexal mass 6.1 X 4.6 X 4.9 cm Tumor Markers : Normal. CLINICAL IMPRESSION :NUT midline carcinoma with Hilar Lymhnodal mass and Ovarian mass Started on Etoposide+Cisplatin in v.o Ovary+Hilar nodal mass considering Germ Cell Tumor w.e.f 30/3/19 . After 2#: Clinically : Decreased cough and SOB. Radiologically : Decrease in size of conglomerate nodal mass 5.9x8 cms---> 2.8x1.7 cms Decreased collapse consolidation and encasement . Near total resolution of lymphangitis carcinomatosis complete resolution of Right pleural efusion . Moderate increase in ovary( midline pelvic mass) size from 6.1x4.6 ---> 9x5.2 cms. Planned to coninue 2#of E+P more followed by Sx Received C#3 w.e.f 15/5/19 to 19/5/19 and C#4 w.e.f 5/6/19 Response assessment : Clinically : Increase in cough and abdominal pain a.w on and off vomiting Radiologically : Increase in size of pelvic mass from 9x5.2 cms---->10.5x5.5cms with new onset freefluid in pelvis.Minimal clumping of small bowel loops is noted s.o adhesions.Nodal mass in chest and collapse consolidation of right middle lobe is stable. Met gynaecologist and adviced to undergo removal of pelvic mass . In v.o clinical and radiological progression adviced weekly Paclitaxel ( completed 3# Paclitaxel) and currently in the process of procuring BET inhibitors from BI Company on compassionate basis.

Clinical trial identification

Editorial acknowledgement