Rare primary mature teratoma of the liver: A case report.

BACKGROUND: Primary liver teratoma is an extremely rare tumor usually affecting children under the age of 3 years. Specific signs of teratoma on ultrasound, computed tomography (CT) or magnetic resonance imaging are lacking, which makes morphology the only diagnostic tool. Misdiagnosis of a mature teratoma may lead to excessive liver resection, whereas misdiagnosis of an immature teratoma may result in spread, causing a life-threatening condition. Consequently, a careful tumor examination is important, and the rarest types of tumors must be accounted for. CASE SUMMARY: We describe a 52 years old female who presented with a solid mass in the left liver lobe. Contrast-enhanced CT and magnetic resonance imaging (MRI) revealed a round, heterogeneous lesion containing a number of fluid areas and areas of calcification in the middle, and the provisional diagnosis was cholangiocarcinoma. The patient underwent resection of liver segment I. Immunohistochemistry analysis of the resected lesion indicated thyroid follicular epithelium; however, the thyroid gland was intact. 10 years prior to presentation the patient underwent a surgery due to mature teratoma of the right ovary, nevertheless the tumor was benign and could not spread to the liver, in addition teratoma of the liver was also benign. This led to the final diagnosis of primary mature liver teratoma. CONCLUSION: Primary hepatic teratoma, including heterotopia of the thyroid gland in the liver, is an extremely rare condition in adults that needs to be considered in the differential diagnosis of solid-cystic neoplasms in the liver and cholangiocarcinoma. This case adds to the limited literature on the patient presentation, clinical workup and management of liver teratomas.

Leiomyosarcoma of the inferior vena cava.

Leiomyosarcoma of the inferior vena cava (IVC) is a rare malignancy. Approximately 350 cases of this disease are known worldwide. Surgical treatment of this condition remains an extremely difficult task. In this case report, we present an experience of successful radical treatment of a leiomyosarcoma of the suprarenal IVC without vascular reconstruction. The patient was treated with resection of the suprarenal portion of the IVC with the tumor; however, in view of adequate blood outflow from the right lobe of the liver, prosthetic replacement of the IVC with right hepatic vein reimplantation was not performed.

Perihilar cholangiocarcinoma: A different concept for radical resection.

BACKGROUND: Long-term outcomes depend heavily on the possibility of performing radical resection. PURPOSE: To evaluate long-term results in perihilar cholangiocarcinoma (PHC) patients from the perspective of a new understanding of radical resection. METHODS: Consecutive PHC patients who underwent surgical resection at A.V. Vishnevsky Center of Surgery from 2011 to 2018 were retrospectively reviewed. Fifty eight (87.9%) patients underwent hemihepatectomy (14 extended hemihepatectomies), while 2 (3%) underwent extrahepatic bile duct resection only, 6 (9.1%) underwent S4b, and 5 underwent en bloc resection of the extrahepatic bile duct. The influence of the bile duct wedge R status, nodal status, microvascular invasion, microlymphatic invasion, perineural invasion, liver invasion, and surrounding adipose tissue invasion on survival was tested by Cox's models. Survival rates depending on pathological parameter numbers were compared by log-rank tests. RESULTS: Wedge resection, nodal status, microvascular invasion, microlymphatic invasion, perineural invasion, liver invasion, and surrounding adipose tissue invasion served as extended criteria for curability (R+, 1 to 7 parameters). For R0 resection status and R1 resection status (R+1, R+2), 7 and 5 parameters were negative, respectively. For R1+ resection status (R+3, R+4, R+5, R+6, R+7), 3 to 7 parameters were positive. Patients who underwent R0 and R1 resections had 5-year survival rates of 100%; the 1- 2- 3-year survival rates were 63%, 49%, 33% for patients who underwent R1+ resections, respectively. The expanded criteria for determining radical resection levels correlated with long-term outcomes (р = 0.0001). CONCLUSION: The new concept for radical resection can accurately reflect surgical treatment results and contribute to selecting appropriate adjuvant therapies in PHC patients. TRIAL REGISTRATION: The study was carried out in accordance with the framework of the "Multimodal treatment of primary and secondary malignant tumors of the liver and pancreas" (state registration № 315030310062).

Personalised approach in combined treatment of cholangiocarcinoma: a case report of healing from cholangiocellular carcinoma at stage IV.

We describe a rare case of healing from low differentiated cholangiocarcinoma stage T4N1M0 with atypical genetic mutation in gene BRAF V600E for this tumor. A 38-year-old female patient was operated in National Surgery Institute due to cholangiocellular carcinoma (CCA) of left liver lobe with anterior abdominal wall invasion, invasion into diaphragm, pericardium. Left liver resection with lymphatic dissection, pericardial resection was performed. Adjuvant chemotherapy (GEMOX) didn't give any results. Treatment with pembrolizumab also didn't result in any improvement. Next generation sequencing molecular tumor profiling revealed mutation in BRAF V600E gene. Target therapy with dabrafenib and trametinib, a BRAF gene inhibitors was initiated and resulted in a full response. The patient is for 2 years tumor free with no signs of recurrence. To our knowledge our case report is longest in the world for stage IV CCA treated with dabrafenib + trametinib.