ABSTRACT
In paediatric population occurrence of ovarian cancer is rare. In this study, an 8-year-old girl was diagnosed mixed malignant ovarian Germ Cell Tumuor (GCT), with predominantly Embryonal Carcinoma component (95%) and focal dysgerminoma component (5%). The patient presented with dull aching pain abdomen and mass in abdomen. On examination mass occupied whole of abdomen, hard in consistency, mobile and lower border per vaginally occupying right fornix. CECT abdomen was done which showed a well-defined solid cystic mass measuring 24.1x 18.7x 14.9 cm mass arising from the right adnexa causing suggestive of malignancy. Uterus, right ovary and rest of the peritoneal cavity was normal. CECT chest was normal. Tumour markers S. Beta-hCG was 27,601.44 mIU/mL, S. LDH was 1735 IU/mL, S. AFP was >400 ng/ mL and S.CA-125 was 114.5IU/mL. After multidisciplinary tumuor board discussion patient was planned for staging ovarian laparotomy (fertility preserving surgery). Intraoperatively ascites was present, right ovary was enlarged measuring about 25x15 cm occupying whole of abdomen, adherent to small bowel, fundus of uterus and right fallopian tube. Left ovary, Fallopian tube and Uterus was normal. Right pelvic and paraaortic lymph nodes was enlarged. Liver, bilateral diaphragm and rest of the peritoneal cavity normal. Procedure done was excision of right ovarian mass with right salpingectomy, bilateral pelvic lymph node dissection, retroperitoneal lymph node dissection, greater omentectomy and peritoneal biopsies. On histopathology right ovary was reported as poorly differentiated neoplasm. In retroperitoneal lymph nodes 1 out 15 lymph nodes showed tumour deposits measuring 0.2cm. Right fallopian tube, bilateral pelvic lymph nodes, greater omentum, peritoneal biopsies and ascitic fluid were free of tumour. On immunohistochemistry (IHC), right ovary tissue was positive for SALL4 and PLAP (germ cell tumour marker). CD30 (Embryonal carcinoma marker) was diffusely positive and CD117 (dysgerminoma marker) was focally positive. It was negative for Beta-hCG (choriocarcinoma marker), AFP (yolk sac tumour marker), EMA (epithelial marker), Calretinin (sex cord stromal tumour marker) and Inhibin (sex cord stromal tumuor marker). From above findings, diagnosis of FIGO stage III A1 (ii) (T1cN1aM0) mixed malignant GCT was made. Patient was advised adjuvant chemotherapy with Bleomycin, Etoposide and Cisplatin (BEP).
ABSTRACT
Primary Hepatic Leiomyosarcoma are very rare Tumours with only 30 cases reported Worldwide1. Patient complains of nonspecific symptoms and often presents with Enlarged Liver. Diagnosis is usually delayed and patients have poor prognosis2. 35-year-old woman presented to Outpatient Department (OPD) complaining of dull aching pain and mass in upper part of abdomen for 6 months. On palpation liver was enlarged and lower border extended up to umbilicus. Ultrasonography of abdomen showed Liver enlarged 23 cm with multiple hypoechoic nodules noted in both lobes of liver. Triphasic Contrast-Enhanced Computed Tomography (CECT) abdomen multiple variable sized Peripherally Enhanced Hypodense Nodules in both lobes of Liver suggestive of Multifocal Hepatocellular Carcinoma (HCC)/ Metastasis. Tumour markers S alpha fetoprotein, S. CA 19-9, S. CEA was within normal limits. CECT Chest was normal. Positron Emission Tomography and Computed Tomography (PET-CT) was done, which showed Liver enlarged 24.5cm with FDG avid multiple nodules in both lobes of Liver and multiple enlarged FDG avid periportal, Portocaval and Peripancreatic Lymph Nodes. Rest of body organs were normal. Biopsy from the Liver showed Mesenchymal Tumour composed of spindle cells arranged in fascicular growth pattern, nucleus cigar shaped with atypia and mitotic figures seen. On Immunohistochemistry (IHC) Tumour was positive markers were Smooth Muscle Actin (SMA), VM, Desmin and H-caldestron and negative for CK, S-100, SOX-10, CD-117 and Dog-1. From above work up a diagnosis of primary Leiomyosarcoma of Liver was made. Palliative Chemotherapy was offered to the patient.