Aleluia, C

Paixão

Penha, D

Pinto, E

Rosado, E

Repositório do Hospital Prof. Doutor Fernando Fonseca

Case 10244 Small cell carcinoma of the ovaryElsa Rosado, Erique Pinto, Diana Penha, Pedro Paixão, Clara Aleluia  Genital (Female) Imaging Section: 2012, Sep. 24 Published: 15 year(s), femalePatient:Authors' InstitutionHosp. Fernando Fonseca; Amadora, Portugal; Email:elsasprr@hotmail.comClinical HistoryA 15-year-old girl, with no relevant personal or medical history was admitted at the emergencydepartment with a large, painless mass in the hypogastric region, extending to the umbilical region.Laboratorial evaluation revealed an elevated calcium level (15, 5 mg/dl) and a decreased phosphorlevel (1, 9mg/dl). Tumour markers were negative.Imaging FindingsPelvic and abdominal ultrasound demonstrated a 12x10 cm heterogeneous pelvic mass, with smoothborders and mixed structure, with solid and cystic areas. The origin of the mass could not bedetermined by ultrasound, however, a relation with the left ovary was suspected. Abdominal organswere normal, except for a slight hydronephrosis in the left kidney.Pelvic MRI showed a median mass with an approximated volume of 600cc, which suggested aprimitive lesion of the left ovary. It had well defined, regular borders, with a peripheral,heterogeneous solid component, and a central cystic area, with multiple thin septa and a mildlyincreased T1 signal without fat suppression, compatible with a protein-mucinous component.Surgery was performed. Pathological examination demonstrated a small cell carcinoma of the ovaryand staging CT revealed no visible metastases. Final tumour staging were stage IA (T1aN0M0).DiscussionSmall cell carcinoma of the ovary is a rare malignant tumour arising primarily in young women,with an average age at presentation of 24 years [1, 2]. The histogenesis of this tumour is unknown,as the tumour cells cannot be subtyped as surface epithelial, germ cell, sex-cord or neuroendocrinecells. It is the most common undifferentiated ovarian carcinoma in young women, and it should beincluded in the differential of ovarian tumours in this age group, along with well differentiatedtumours [2, 3]. Clinical presentation includes pelvic mass, abdominal pain, nausea, vomiting, menstrualirregularities and infertility. Approximately two thirds of patients with ovarian small cell carcinomahave hypercalcemia. Among those patients, only a minority have symptoms related to thehypercalcaemia, such as hypertension, fatigue, polydipsia, obstipation, abdominal and lumbar pain,as well as renal insufficiency [3].MRI is the method of choice for imaging pelvic masses. Images must be obtained in two or moreplanes. Acquisition of both T1 and T2 weighted images and fat-saturated T1 images are essential. Inthis case, the increased T1 signal intensity of the lesion could represent fat, haemorrhage or mucin.As the lesion didn't lose signal intensity after fat saturation, fat could be excluded, andendometriomas and hemorrhagic cysts are often dark on T2. High viscosity mucin can be bright onT1 weighted images, whether low viscosity mucin is dark. Combining image findings on T1, T2and fat saturation sequences, a mucinous content of the lesion could be suspected.Gadolinium-enhanced T1-weighted images could help characterise the internal architecture of thecystic components [4, 5].There are no specific MRI signal intensity characteristics for most malignant tumours. Thus,surgery is mandatory when a suspicious adnexal mass is found. Concerning small cell carcinoma ofthe ovary, therapy is primarily surgical, with adjuvant treatment reserved for advanced-stage disease[2].Small cell carcinoma of the ovary has a poor prognosis. The most important prognostic factor is thestage of the tumour at the time of diagnosis. More than 50% of stage IA patients die within 2 yearsand 33% have an average 6-year disease free survival. Among the stage IA group, patients withtumour size less than 10 cm have a better prognosis, compared with those with larger tumours [2,3].Ovarian small cell carcinoma is a rare malignant tumour with poor outcome. Young age atpresentation, hypercalcemia and imaging findings are essential diagnostic features.Final DiagnosisSmall cell carcinoma of the ovaryDifferential Diagnosis ListMucinous cystadenoma / cystadenocarcinoma, Serous cystadenoma / cystadenocarcinoma,Endometrioide carcinoma, Clear cell carcinoma, Teratoma, Dysgerminoma, Endometrial sinustumour, Embryonal carcinoma, Granulosa cell tumour (juvenile type), Sertoli-Leydig cell tumourFiguresFigure 1 Abdominal and pelvic ultrasoundLongitudinal plane in the pelvis demonstrating a heterogeneous mass with smooth bordersand mixed structure, containing solid and cystic areas. © Rosado E, Department of Radiology, Hospital Fernando Fonseca, Amadora, Portugal Area of Interest: Genital / Reproductive system female;  Imaging Technique: Ultrasound;  Procedure: Diagnostic procedure;  Special Focus: Neoplasia; Transversal plane in the pelvis demonstrating a heterogeneous pelvic mass with smoothborders and mixed structure, containing solid and cystic areas. © Rosado E, Department of Radiology, Hospital Fernando Fonseca, Amadora, Portugal Area of Interest: Genital / Reproductive system female;  Imaging Technique: Ultrasound;  Procedure: Diagnostic procedure;  Special Focus: Neoplasia; During the abdominal scan, a moderated hydronefrosis was noted in the left kidney © Rosado E, Department of Radiology, Hospital Fernando Fonseca, Amadora, Portugal Area of Interest: Kidney;  Imaging Technique: Ultrasound;  Procedure: Diagnostic procedure;  Special Focus: Obstruction / Occlusion; Figure 2 Abdominal and pelvic MRIAxial T1: primitive lesion of the left ovary with well defined, regular borders. © Rosado E, Department of Radiology, Hospital Fernando Fonseca, Amadora, Portugal Area of Interest: Genital / Reproductive system female;  Imaging Technique: MR;  Procedure: Diagnostic procedure;  Special Focus: Neoplasia; Axial T2: primitive lesion of the left ovary containing a peripheral heterogeneous solidcomponent and a central cystic area, with multiple thin septa. © Rosado E, Department of Radiology, Hospital Fernando Fonseca, Amadora, Portugal Area of Interest: Genital / Reproductive system female;  Imaging Technique: Ultrasound;  Procedure: Diagnostic procedure;  Special Focus: Neoplasia; Sagittal T2: primitive lesion of the left ovary containing a peripheral heterogeneous solidcomponent and a central cystic area, with multiple thin septa. © Rosado E, Department of Radiology, Hospital Fernando Fonseca, Amadora, Portugal Area of Interest: Genital / Reproductive system female;  Imaging Technique: Ultrasound;  Procedure: Diagnostic procedure;  Special Focus: Neoplasia; Coronal T2: primitive lesion of the left ovary containing a peripheral heterogeneous solidcomponent and a central cystic area, with multiple thin septa. © Rosado E, Department of Radiology, Hospital Fernando Fonseca, Amadora, Portugal Area of Interest: Genital / Reproductive system female;  Imaging Technique: Ultrasound;  Procedure: Diagnostic procedure;  Special Focus: Neoplasia; Figure 3 Abdominal and pelvic MRIAxial T2 fat saturation image demonstrating that the lesion contains no fat, but probably aproteic-mucionous material. © Rosado E, Department of Radiology, Hospital Fernando Fonseca, Amadora, Portugal Area of Interest: Genital / Reproductive system female;  Imaging Technique: Ultrasound;  Procedure: Diagnostic procedure;  Special Focus: Neoplasia; Sagittal T2 fat saturation image demonstrating that the lesion contains no fat, but probably aproteic-mucionous material. © Rosado E, Department of Radiology, Hospital Fernando Fonseca, Amadora, Portugal Area of Interest: Genital / Reproductive system female;  Imaging Technique: Ultrasound;  Procedure: Diagnostic procedure;  Special Focus: Neoplasia; Figure 4 Abdominal and pelvic MRIAxial T2: normal right ovary (yellow arrow). © E Rosado, Department of Radiology, Hospital Fernando Fonseca, Amadora, Portugal Area of Interest: Genital / Reproductive system female;  Imaging Technique: Ultrasound;  Procedure: Diagnostic procedure;  Special Focus: Neoplasia; Sagittal T2: normal right ovary (yellow arrow). © E Rosado, Department of Radiology, Hospital Fernando Fonseca, Amadora, Portugal Area of Interest: Genital / Reproductive system female;  Imaging Technique: Ultrasound;  Procedure: Diagnostic procedure;  Special Focus: Neoplasia; Figure 5 Abdominal and pelvic MRIAxial T2: the mass is causing homolateral ureterohydronephrosis (yellow arrow). © E Rosado, Department of Radiology, Hospital Fernando Fonseca, Amadora, Portugal Area of Interest: Genital / Reproductive system female;  Imaging Technique: Ultrasound;  Procedure: Diagnostic procedure;  Special Focus: Neoplasia; MeSH [A05.360.319.114.630]OvaryThe reproductive organ (GONADS) in female animals. In vertebrates, the ovary contains twofunctional parts: the OVARIAN FOLLICLE for the production of female germ cells(OOGENESIS); and the endocrine cells (GRANULOSA CELLS, THECA CELLS, and LUTEALCELLS) for the production of ESTROGENS and PROGESTERONE. [C04.588.945.418.685]Ovarian NeoplasmsTumors or cancer of the OVARY. These neoplasms can be benign or malignant. They are classifiedaccording to the tissue of origin, such as the surface epithelium, the stromal endocrine cells, and thetotipotent germ cells.References[1] 1. Dickersin GR, Kline IW, Scully RE (1982) Small cell carcinoma of the ovary withhipercalcemia: report of eleven cases. Cancer 49:188-97[2] Chen L, Din TA, Haque A (2005) Small cell carcinoma of the ovary with hypercalcemia andectopic parathyroid hormone production. Arch Pathol Lab Med. 129(4):531-3[3] Young RH, Oliva E, Scully RE (1994) Small cell carcinoma of the ovary, hypercalcemic type. Aclinicopathological analysis of 150 cases. Am J Surg Pathol. 18(11):1102-16[4] Jeong YY, Outwater EK, Kang HK (2000) Imaging evaluation of ovarian masses.Radiographics 20(5):1445-70[5] Jung SE, Lee JM, Rha SE, Byun JY, Jung JI, Hahn ST (2002) CT and MR imaging of ovariantumors with emphasis on differential diagnosis. Radiographics 22(6):1305-25CitationElsa Rosado, Erique Pinto, Diana Penha, Pedro Paixão, Clara Aleluia (2012, Sep. 24)  Small cell carcinoma of the ovary {Online}URL: http://www.eurorad.org/case.php?id=10244

Small cell carcinoma of the ovary

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Small cell carcinoma of the ovary

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