Atypical 18F-FDG PET-CT Findings in a Rare Case of Primary Hepatic Leiomyosarcoma.

The primary hepatic leiomyosarcoma is a rare malignant tumor arising from the smooth muscle cells in the hepatic vessels, bile ducts, and ligamentum teres. It is considered a subtype of hepatic sarcomas. We report awkward 18F-FDG PET-CT findings of a primary hepatic leiomyosarcoma masquerading as a benign hepatic tumor, which were confirmed by histopathological and immunohistochemical examinations in a 78-year-old woman.

Not all liver tumors are alike - an accidentally discovered primary hepatic leiomyosarcoma: A case report.

BACKGROUND: Primary hepatic leiomyosarcoma is a very rare entity that originates from smooth muscle. Preoperative diagnosis requires a high degree of suspicion due to atypical clinical presentation and non-specific imaging features. CASE SUMMARY: We report the case of a 42-year-old man, with no relevant past medical history, accidentally diagnosed with a nodular liver lesion on a routine abdominal ultrasound. Liver function tests and hematology parameters as well as tumor markers were normal. A contrast-enhanced abdominal computed tomography scan revealed a heterogenous hepatic lesion measuring 40 mm 30 mm, adjacent to the left branch of the portal vein and the round ligament. Due to the unclear diagnosis, the patient underwent surgical resection. Histopathological and immunohistochemical examinations confirmed complete (R0) resection of a hepatic leiomyosarcoma. The patient remains without any signs of tumor recurrence for more than 2 years. CONCLUSION: We report a rare case of accidentally diagnosed primary hepatic leiomyosarcoma originating from the portal vein or the round ligament. Although this tumor has aggressive metastatic potential, a tumor-free resection margin is essential to improve survival.

Primary hepatic leiomyosarcoma successfully treated by transcatheter arterial chemoembolization: A case report.

BACKGROUND: Primary hepatic leiomyosarcoma is rare and reported sporadically, with less than 40 such cases have been reported in the English-language literature. Although it is reported to be associated with acquired immune deficiency syndrome, Epstein-Barr virus infection, Hodgkin's lymphoma, immunosuppression after organ transplantation, and hepatitis C virus-related liver cirrhosis, the precise steps leading to leiomyosarcoma have not been fully identified. Therapeutic strategies include liver wedge resection or lobectomy, chemotherapy, radiotherapy and liver transplantation; however, the prognosis of primary hepatic leiomyosarcoma is dismal. CASE SUMMARY: We describe here the first case of primary hepatic leiomyosarcoma successfully treated by transcatheter arterial chemoembolization (TACE). The patient was a 68-year-old woman who presented with right upper quadrant pain and weight loss over the past 5 wk before admission. Abdominal computed tomography (commonly known as CT) and ultrasonography showed a mixed echoic mass measuring about 10 cm × 7 cm occupying the right lobe of the liver. Exploratory laparotomy was performed 1 wk after admission. The tumor was unresectable and biopsy was performed. Based on rapid frozen-section and histopathological examination, a final diagnosis of primary hepatic leiomyosarcoma was established. TACE was performed 2 wk later. The postoperative course was uneventful and the patient was discharged on day 7 after the operation. Contrast-enhanced CT showed that the tumor significantly shrunk with satisfactory lipiodol deposition. The patient has been followed up for 82 mo until now, and no progressive enlargement of the tumor or distal metastasis was observed. CONCLUSION: TACE is a safe and effective treatment for primary hepatic leiomyosarcoma. The therapeutic effect of TACE combined with surgical resection should be further assessed.

Primary Hepatic Leiomyosarcoma: Histopathologist's Perspective of a Rare Case.

Primary hepatic leiomyosarcoma is a rare primary mesenchymal tumor of the liver requiring exclusion of any other primary site of origin and histological and immunohistochemical exclusion of other hepatic/extrahepatic tumors with spindle cell morphology. Only about 70 cases are reported in the English literature and many of these tumors have predisposing conditions in the form of immunosuppression or associated malignancies. The occurrence of this tumor in the immunocompetent individual is also known. Histomorphology of this tumor shows a spindle cell lesion which needs to be distinguished from other spindle cell lesions of this region. The main diagnostic challenge of this tumor lies in its rarity, lack of awareness and morphological mimickers in the given site. A complete range of immunohistochemical markers is required to distinguish the lesion from its close morphological mimickers. Here, we discuss a case of primary hepatic leiomyosarcoma in an adult female patient with detailed histomorphological differentials and respective immunoprofiles.

Primary gigantic leiomyosarcoma of the liver treated with portal vein embolization and liver resection.

Primary hepatic leiomyosarcoma (PHL) is an extremely rare tumor. The tumor has no specific presentations and often diagnosis is delayed until it reaches a significant size. We report the case of a 69-year-old female presented with a huge PHL. Due to size of the tumor and to be operable, the patient subjected to right portal vein embolization (PVE) and selective embolization of segment V. Four weeks after the PVE, liver resection was conducted (Segments V+VI bisegmentectomy plus resection of IVA). The patient had an uncomplicated post-operative course, and discharged at the 8th post-operative day. Diagnosis of PHL was confirmed by histopathological and immunohistochemical examinations. The patient refused to receive adjuvant chemotherapy, and revealed evidence of recurrence six months after the operation, and finally died 12 months after the operation and 16 months after initial diagnosis. PHL is an extremely rare tumor and often in first presentation has significant size. Radical surgery with adjuvant chemotherapy is key feature for prolonged survival.

Primary hepatic leiomyosarcoma in a patient with autosomal dominant polycystic kidney disease.

Primary hepatic leiomyosarcoma is an extremely rare tumor. The diagnosis is difficult, and its etiologic factors have not been clarified. A 63-year-old woman with numerous cysts in her kidneys and liver was diagnosed with autosomal dominant polycystic kidney disease (ADPKD). Several members of her family also had ADPKD. She underwent treatment with tolvaptan to inhibit cyst growth and slow the decline in kidney function. Eight months after the start of the therapy, she was hospitalized with fatigue and fever of unknown origin. Diagnostic imaging showed a very large hepatic tumor, and histologic examination of a fine-needle biopsy specimen revealed the tumor to be malignant. Differentiation between carcinoma and sarcoma was difficult based on the histological findings. The tumor was thought to be excisable; therefore, hepatic resection was attempted. At the time of surgery, as the tumor had grown larger than when imaged, complete resection was impossible. However, a part of the tumor was resected. Histopathological and immunohistological examinations of the surgical specimen confirmed a primary hepatic leiomyosarcoma. Whether the tumor was associated with the presence of ADPKD remains unclear, however, this is the first report of the combination of these two diseases in a patient.

Imaging features of primary hepatic leiomyosarcoma: A case report and review of literature.

Primary hepatic leiomyosarcoma (PHL) is an extremely rare tumour. This tumour is difficult to diagnose by imaging examinations due to its rarity, and non-specific conventional imaging manifestations and clinical presentation. The present study reports the case of a 42-year-old male with PHL that was confirmed by histopathological and immunohistochemical examinations. Multimodal imaging examinations, including ultrasound, computed tomography (CT), magnetic resonance imaging (MRI), positron emission tomography-CT and digital subtraction angiography, were performed. The imaging manifestations were analysed and the associated literature was reviewed. The results found that no characteristic imaging appearance was present on ultrasound or plain CT scan. However, on unenhanced MRI, the tumours presented with a heterogeneous low signal density on T1-weighted imaging (WI) and a high signal density on T2WI and diffusion-WI. On gadopentetate dimeglumine enhanced MRI, the lesions were not enhanced during the arterial and portal venous phases; by contrast, these lesions were evidently enhanced during the 5-min delayed phase. Therefore, the delayed imaging of enhanced MRI is likely to be used to differentiate PHL from other hepatic tumours.

Primary hepatic leiomyosarcoma: Case report and literature review.

Primary hepatic leiomyosarcoma are rare tumors with less than 30 cases reported in the English literature. Non specific presentations and often diagnosis delayed until they reach a large size, is the norm with therapy leading to an often dismal prognosis. A 67-year-old man presented complaining of abdominal pain and a palpable abdominal mass since Jan 2010. Abdominal ultrasonography and abdominal computed tomography revealed a large tumor in the left lobe of the liver. Surgical exploration was undertaken and an extended left hepatectomy with extension onto the dorsal part of segment 8 preserving the MHV with partial resection of segment 6 was undertaken. The weight of the resected specimen was 1300 g of the left lobectomy specimen and 8 g of the segment 6 partial resection specimen. The pathology report confirmed the diagnosis of leiomyosarcoma. On immunohistochemistry, the tumor cells were positive for smooth muscle actin stain. The patient is on regular follow up and is currently 9 mo post resection with no evidence of recurrence. We report the case of a resected primary hepatic leiomyosarcoma and emphasize the need for a global database for these rare tumors to promote a better and broader understanding of this less understood subject.

Primary Hepatic Leiomyosarcoma: A case report

The liver is a distinctly unusual locus for a primary leiomyosarcoma. In October 1994, a 64-year-old female patient was admitted with palpable mass at the right upper abdomen for 2 months. Ultrasonography and Computed Tomography revealed a big cystic nature tumor at the right lobe of the liver. A right lobectomy was undertaken. On gross examination of the specimen, the tumor size was measured a 7.5 cm 16.0 cm 4.5 cm. On a cut section, cystic degeneration was showed and the color of the solid component was grayish. Microscopic examination growth pattern of the tumor to be expansive with no capsule. Finally the leiomyosarcoma of the liver was diagnosed with 5 to 6 mitosis per 10 high power fields. In September 1995, follow-up Computed Tomography revealed a recurred mass on segment 4 of the liver. A tumonectomy was performed. Twenty-one months after the second operation, recurrence was noted on the remaining liver and intraabdomen. Debulking surgery of the intraabdominal mass with intraperitoneal chemotherapy was undertaken. We report this case of primary hepatic leiomyosarcoma with a review of the literatures.