small cell carcinoma of the ovary of hypercalcemic type ( sccoht ) is a very rare tumor usually found in young females . it is a highly malignant tumor with a poor prognosis [ 1 , 2 , 3 ] . based on a similar genetic profile to malignant rhabdoid tumors , it was suggested to use the term  malignant rhabdoid tumor of the ovary [ 4 , 5 ] . herein , we present a recent case of this rare entity from our practice and discuss its differential diagnosis and management . a 24-year - old female was referred by her community - based gynecologist with abdominal pain lasting for over 3 weeks and a feeling of bloatedness . on physical examination diagnostic ultrasound confirmed an isoechogenic , nonhomogeneous , rounded solid and cystic mass of 20 cm in diameter . slight elevation of liver enzymes and blood urea nitrogen was detected ; otherwise , blood tests were within normal limits . the tumor marker ca125 was elevated at 59.5 kiu / l [ upper limit of normal ( uln ) : 35 kiu / l ] , he4 at 201.8 pmol / l ( premenopausal uln : 70 pmol / l ) , and the corresponding roma ( risk of ovarian malignancy algorithm ) index was 70.77% . contrast - enhanced ct examination demonstrated a cystic tumor with some solid components arising from the right ovary . the tumor measured 20 cm in diameter ; there was a minimal amount of free fluid . there were no other pathological findings on ct examination ( fig .  1 ) . during diagnostic laparotomy , we found a large tumor mass filling the true pelvis and abdominal cavity up to and above the umbilicus . the tumor was completely resected and sent for histopathological examination . grossly , the tumor measured 16 cm ( after formalin fixation ) , and it showed a soft consistency , an incomplete and thin pseudocapsule , and gray - to - yellowish cut surface with necrosis and hemorrhages . 2 ) , it was composed of small cells with high nuclear atypia and numerous mitoses . the cell population was solid , with follicle - like spaces and with some areas showing a trabecular pattern . the tumor cells focally penetrated the fibrous pseudocapsule , and rare foci of the vascular invasion were seen . immunohistochemically , the tumor was diffusely positive for p53 and wt1 ( see online suppl . the following antibodies gave negative results : estrogen and progesterone receptors , -inhibin , calretinin , oct3/4 , c - kit , placental alkaline phosphatase , leukocyte common antigen , s-100 protein , hmb45 , melan a , chromogranin a , cd56 , synaptophysin , -smooth muscle actin , desmin , h - caldesmon , fli-1 , and neurofilament protein . it was decided to perform a radical oncological staging procedure , but to leave the uterus , for possible future pregnancy through oocyte donation . the findings on the patient 's ovaries , greater omentum , appendix , peritoneal biopsies , 15 and 24 paraaortic and pelvic lymph nodes , respectively , were all without malignancy . at this stage , 400 ml of ascites was detected , which was cytologically negative for tumor cells . after uneventful recovery , she underwent 4 cycles of adjuvant chemotherapy with cisplatin and etoposide . during this time , the patient 's calcium and tumor markers ( ca125 and nse ) were within normal limits . a ct scan at completion of the adjuvant chemotherapy was without any recurrence . four months after the final cycle of adjuvant chemotherapy , the patient was stared on hormone replacement treatment . five months after completion of the adjuvant chemotherapy , she presented with anorexia , generalized weakness , nausea , and constipation . during physical examination , deep palpation in her right hypogastric region revealed a poorly defined , nontender mass . a staging ct scan revealed 2 tumors in the abdominal cavity : the first was placed above the aortic bifurcation with a dimension of 81  47  106 mm ( ap  ll  cc ) and causing compression of the inferior vena cava and the surrounding structures ; the second one was in the true pelvis measuring 95  66  60 mm ( fig . malignant hypercalcemia was detected with a blood calcium of 5.26 mmol / l ( uln : 2.7 mmol / l ) and with corresponding shortening of the qt interval ( 240 ms ) on ecg . after the second cycle of this palliative treatment , she was urgently operated for ileus . during this third surgery , the 2 tumor masses were visualized : the first was retrocecal , and the second one filled the whole true pelvis with infiltration of the urinary bladder . unfortunately , the patient died on the 4th postoperative day with signs of massive pulmonary embolism ( 10 months after initial surgery ) . this rare tumor entity was first fully described by dickersin et al .   in 1982 . before this the clinical picture of sccoht includes young age , with a median of 2428 years , hypercalcemia in two thirds of cases , and a stage higher than i in 75% of patients . our young female patient had initial normocalcemia , and the tumor markers were nonspecifically raised . the histological diagnosis of sccoht is based on the finding of a small cell population with high nuclear atypia , numerous mitoses , and frequent necrosis . the growth pattern is usually solid and trabecular , with typical and almost pathognomic follicle - like spaces . in addition , some unusual findings have been described , such as mucinous glands , mucinous signet ring cells , spindle cell sarcomatoid change , large cells , and rhabdoid cytomorphology [ 1 , 3 ] . immunohistochemically , sccohts are positive for vimentin , and they show a focal expression of epithelial markers . in addition , expressions of p53 , cd56 , wt1 , calretinin , cd10 ,  parathyroid - related polypeptide , and -smooth muscle actin were described [ 3 , 8 ] . in cases with rhabdoid cytomorphology , molecular genetic studies showed that occasional rhabdoid morphology is quite in accordance with genetic changes . sccohts contain alterations in genes smarcb1 or smarca4 , which are frequent also in malignant rhabdoid tumors . this view is supported by the following features shared by both sccoht and malignant rhabdoid tumor : occasional familial occurrence , frequent hypercalcemia , and immunohistochemical polyphenotypia [ 3 , 4 , 8 ] . it remains , however , to explain some findings which are not typical for rhabdoid tumor , such as mucinous glands , the common absence of rhabdoid cells , and findings of smarc4 alterations in some neoplasms unrelated to rhabdoid tumor ( pulmonary and pancreatic carcinomas , endometrioid carcinomas ) [ 10 , 11 ] . adjuvant chemotherapy with cisplatin and etoposide usually follows ; some authors also recommend external beam radiotherapy . this treatment scheme is commonly employed in small cell lung cancer and in small cell cancers generally . most patients with sccoht respond to chemotherapy treatment employing a combination of agents , such as cisplatin , etoposide , doxorubicin , bleomycin or cyclophosphamide and vinblastine . some authors recommend fertility - sparing surgery followed by adjuvant chemotherapy , others suggest radical staging surgery with pelvic and paraaortic lymphadenectomy [ 13 , 14 ] . for advanced stages , neoadjuvant chemotherapy , followed by radical staging surgery is recommended . due to the highly aggressive nature of this tumor only a third of early - stage patients have the benefit of a long relapse - free interval . these patients are usually older than 30 years , have normal blood calcium , their tumor mass is < 10 cm without large cells on pathology , and they underwent radical staging procedure . in advanced cases , aggressive surgical management , combined with chemotherapy with or without radiotherapy is based only on case reports [ 2 , 15 ] . despite various radical approaches , the 1-year survival of sccoht patients is only around 50% , similar to the case presented above . in the face of these poor results and high morbidity , an aggressive approach to unfortunately , our patient died 10 months after diagnosis , highlighting the highly malignant nature of this tumor . consent for publication of this case report was obtained from the next of kin of the deceased patient .
we present a rare case of malignant rhabdoid tumor ( ovarian small cell carcinoma of hypercalcemic type ) in a 24-year - old female with fulminant course . 
 clinically , hypercalcemia was not found at the time of primary diagnosis . 
 however , it appeared later during the course of tumor progression . 
 histologically , the tumor showed classical features of small cell carcinoma of hypercalcemic type . 
 therapy included radical surgery with adjuvant chemotherapy . despite this intensive therapy , 
 the disease recurred and the patient died 10 months after the diagnosis . 
 we discuss the diagnosis and therapy of this tumor , as well as its recent classification as malignant rhabdoid tumor .