Primary Hepatic Leiomyosarcoma Research Articles

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.

Primary Hepatic Leiomyosarcoma (PHL) with pulmonary metastatic activity. Case report and literature review

Primary Hepatic Leiomyosarcoma (PHL) with pulmonary metastatic activity. Case report and literature review

Primary hepatic leiomyosarcoma masquerading as focal nodular hyperplasia of liver: A wolf in sheep's clothing.

Primary hepatic leiomyosarcoma is a rare hepatic malignancy which requires exclusion of other primary site of origin. Clinical presentation and imaging of this tumor is nonspecific and mimics many other hepatic neoplasms. A 62-year-old female patient presents here with right hepatic mass with insidious onset and radiological features favoring a benign solid lesion suggestive of focal nodular hyperplasia. On right hepatectomy, an encapsulated mass identified about 11 cm in maximum dimension with pushing margin and central scar-like area. Histopathological examination reveals a spindle cell tumor and panel of immunohistochemical markers is required to distinguish it from other morphological mimickers. Diagnosis of primary hepatic leiomyosarcoma requires histopathology along with immunohistochemical examination. It is thus advisable to do preoperative biopsy with immunohistochemistry in all patients having atypical imaging and clinical features.

A rare primary liver tumour case report: 53-year-old man with primary hepatic leiomyosarcoma

A rare primary liver tumour case report: 53-year-old man with primary hepatic leiomyosarcoma

Primary Hepatic Leiomyosarcoma: Case Report and Literature Review

Introduction: Primary hepatic leiomyosarcoma (PHL) is an extremely rare tumor, accounting for 0.2–2% of all primary hepatic malignancies, with less than 70 cases reported worldwide. Herein, we report a case of a 48-year-old female patient with a large surgically resected PHL.

A Unique Case of the Transformation of a Hepatic Leiomyoma into Leiomyosarcoma with Pancreatic Metastases: Review of the Literature with Case Presentation

Primary hepatic leiomyoma (PHL) is a rare entity, with very few cases reported in the literature. Even more rarely, until now practically undescribed, is the transformation of a hepatic leiomyoma into leiomyosarcoma with pancreatic metastases. Here, we report a single case of the progression of PHL in primary hepatic leiomyosarcoma, with clinical–surgical and histopathological features, and we conducted a review of the literature of related cases that can be found.

多次化学療法を施行した原発性肝平滑筋肉腫の一例

多次化学療法を施行した原発性肝平滑筋肉腫の一例

Primary hepatic leiomyosarcoma (PHLS): A case report and literature review

We encountered a rare case of HLMS.US and plain CT showed ill-defined para-caval hepatic tumor in the caudate lobe. The contrast enhanced CT showed a relatively poorly enhanced tumor with non-enhanced area suggesting necrosis. On MRI, the tumor presented with low signal intensity on T1 in-phased image and heterogeneously high signal intensity on T2WI. On T1-opposed-phase, the tumor showed the slight elevation of the signal compared with T1 in -phased image. This phenomenon suggested shortening of T2* effects caused by faint hemorrhage .On dynamic enhanced MRI, the lesion showed gradually mild enhancement from the arterial to the venous phase with some necrotic area. By the CT-guided biopsy, the diagnosis of leiomyosarcoma with faint hemorrhage was made on immunohistochemical stain.

A case of primary hepatic leiomyosarcoma

A case of primary hepatic leiomyosarcoma

Primary hepatic leiomyosarcoma presenting as a thick-walled cystic mass resembling a liver abscess

Rationale:Hepatic leiomyosarcoma is a rare malignant tumor, and in rare instances, its combination with fever may lead to misdiagnosis of liver abscess. In our case, the presence of the tumor was considered by the presence of blood vessels through the observation of hepatic tumor wall.Patient concerns:A 38-year-old woman who had been diagnosed with a liver abscess in a local hospital consulted our institution by complaining left upper quadrant pain and fever. The diagnosis was questioned in our workup, since no obvious inflammatory marker was found in the circulation, and contrast-enhanced computed tomography revealed a huge thick-walled mass in the right liver with multiple tortuous vessels within the wall.Diagnosis:Imaging revealed a neoplastic lesion rather than liver abscess. The enhancement of the solid tumor tissues was reduced during the portal phase and delayed phase, which was consistent with a wash-out enhancement. The histopathologic and immunohistochemical results confirmed the diagnosis was primary hepatic leiomyosarcoma. A pulmonary nodule during the postoperative follow-up was diagnosed as metastatic leiomyosarcoma tumor through surgery.Interventions:A right hepatectomy was performed to cure the hepatic mass. In the follow-up, a metastatic tumor in the lung was resected, followed by chemotherapy.Outcomes:Lung metastasis and liver recurrence occurred at 25 and 39 months after liver surgery, respectively. Then the patient was lost to follow-up.Lessons:Leiomyosarcoma is associated with a dismal prognosis, but outcomes for these patients may be improved by the combined surgical resection and chemotherapy.

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 Hepatic Leiomyosarcoma Presenting as a Budd-Chiari-Like Syndrome

Figure: Spiral CT scan of the abdomen and pelvis demonstrating a large heterogenous mass occupying the majority of the right hepatic lobe and extending into the IVC.Figure: MRI of the abdomen demonstrating a large cystic and solid mass centered in the right hepatic lobe which extends into the caudate lobe causing near occlusion of the intrahepatic IVC.Primary hepatic leiomyosarcoma is a rare malignant tumor arising from smooth muscle cells of either vascular, biliary, or ligamentous origin. Diagnosis is often delayed due to the tumor causing nonspecific symptoms and often remaining asymptomatic until mass effect is produced. Less than 50 cases have been described in the literature. A 60-year-old male with extensive travel history to developing countries initially presented to a medical facility in Guam with bilateral lower extremity edema, dyspnea, fever, and fatigue. This was attributed to an upper respiratory infection and he was given a course of antibiotics and subsequently developed a diffuse desquamating rash felt to be a drug allergy. He ultimately underwent CT of the abdomen and pelvis which revealed a 13.6 x 16.8cm right hepatic lobe mass with obstruction of the inferior vena cava (IVC). He left Guam and presented to our institution two days later. Due to leukocytosis and fever, the initial suspicion was still largely infectious with hepatocellular carcinoma also on the differential; however he had no history of cirrhosis, hepatitis C, or alcohol abuse. CT guided liver biopsy was performed and demonstrated nonspecific fibrotic and infarcted liver parenchyma. As infectious and tumor markers were unrevealing, a repeat biopsy was obtained. This revealed a poorly differentiated carcinoma without a definitive primary site. Further imaging studies to assess for additional lesions were unrevealing. After extensive discussion with surgical oncology and cardiothoracic surgery he underwent open right hepatic lobectomy with resection of tumor and repair of the IVC. The patient tolerated surgery remarkably well and was subsequently discharged home with plans for potential adjuvant treatment. Surgical pathology, reviewed by an outside institution, demonstrated epithelioid elements with regions of smooth muscle morphology supporting the overall impression of high-grade primary hepatic leiomyosarcoma. Hepatic leiomyosarcoma is an exceedingly rare liver tumor with a poor prognosis due to the characteristically delayed presentation due to nonspecific symptoms. This case is consistent with the presentation and disease course described by previous case reports in the literature. This is thought to be a primary leiomyosarcoma of the liver, most of which are thought to arise from the wall of major vessels. Surgical resection is the management of choice with the role of chemotherapy and radiation not well defined.

Primary Hepatic Leiomyosarcoma A Space Occupying Lesion in the Liver: An Enigma for Diagnosis

Primary hepatic leiomyosarcoma are rare soft tissue sarcomas with about only about 60 cases reported in the English literature [1]. Due to its rare existence and less studied, the diagnostic algorithms and standards of care have not been adequately defined. This has led to a delayed diagnosis and subsequently a poor prognosis and survival of such patients. We herein present a 72 year old lady with primary hepatic leiomyosarcoma treated with chemotherapy and attempt to delineate the line of management after reviewing the medical literature. Primary Hepatic Leiomyosarcoma is a rare primary hepatic malignancy which is usually a diagnosis of exclusion. The standard treatment guidelines are yet to be established; large cohort database is needed for better understanding of disease behaviour and management.

FDG PET/CT Findings of Primary Hepatic Leiomyosarcoma in an Immunocompetent Pediatric Patient

Primary hepatic leiomyosarcoma generally occurs only in adult patients or pediatric patients with immunodeficiency. We report here the FDG PET/CT findings of primary hepatic leiomyosarcoma in a 10-year-old immunocompetent girl.

PRIMARY HEPATIC LEIOMYOSARCOMA REPORT OF A RARE CASE WITH REVIEW OF LITERATURE

Primary hepatic leiomyosarcoma is an extremely rare tumor with a dismal prognosis and difficulty in diagnosis. We present a 36-year-old female who presented with complaints of pain in right hypochondrium and epigastric region. Real-time ultrasonography revealed an enlarged liver with multiple hypoechoic lesions of varying sizes in both the lobes of the liver. USG guided core biopsy from the lesion showed an infiltrating malignant spindle cell neoplasm positive for smooth muscle actin and caldesmon-H confirming the diagnosis of leiomyosarcoma. It is vital to diagnose these lesions even on limited biopsies as early diagnosis can reduce hospital and operative morbidity and mortality rates in the patients. Keywords: Primary hepatic Leiomyosarcoma, small biopsy, immunohistochemistry.

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.

PRIMARY HEPATIC LEIOMYOSARCOMA - A CASE REPORT WITH REVIEW OF LITERATURE

Primary hepatic leiomyosarcoma are very rare tumours with less than 50 cases reported. Due to nonspecific presentations, diagnosis is often delayed until they reach a large size. The rarity of these tumours has precluded our understanding of them and therefore the standard of care has not been well defined. We report a 45-year-old lady who presented with loss of appetite, abdominal distension and bilateral lower limb swelling and hepatomegaly. CT abdomen revealed nodular hepatomegaly with a hypodense lesion on plain scans, heterogenous enhancing lesion on arterial phase and delayed washout on portal venous phase occupying segments VII and VIII and involving right hepatic vein and infiltrating into inferior vena cava (IVC). The pathology report confirmed the diagnosis of leiomyosarcoma. IHC was positive for SMA, vimentin and weakly positive for desmin stain. Due to extensive nature and involvement of IVC, patient was planned for upfront neo-adjuvant chemotherapy (NACT) (Ifosfamide, and doxorubicin with MESNA. The patient has completed 6 cycles NACT and had stable disease. The patient was then switched to oral Pazopanib and is on follow up. Keywords: Primary hepatic leiomyosarcoma, neoadjuvant chemotherapy, unresectable cases

Primary hepatic leiomyosarcoma, case report and review of literature

Primary hepatic leiomyosarcoma, case report and review of literature

Contemporary management and classification of hepatic leiomyosarcoma

BackgroundHepatic leiomyosarcomas are rare soft-tissue tumours. The majority of lesions previously considered as leiomyosarcomas have been identified as gastrointestinal stromal tumours (GISTs). Consequently, understanding of the role of liver resection for true leiomyosarcoma is limited, a fact that is exacerbated by the increasing recognition of leiomyosarcoma subtypes. This study presents data on the outcomes of liver resection for leiomyosarcoma and suggests an algorithm for its pathological assessment and treatment. MethodsPatients were identified from a prospectively collected departmental database. All tumours were negative for c-kit expression. Immunohistochemistry was performed to identify the presence of oestrogen or progesterone receptor (OR/PR) expression or Epstein–Barr virus (EBV) and patients were stratified according to this profile. ResultsEight patients (of whom seven were female) underwent a total of 11 liver resections over a 12-year period. One patient had a primary hepatic leiomyosarcoma. Of those with metastatic leiomyosarcomas, the primary tumours were located in the mesentery, gynaecological organs and retroperitoneum in four, two and one patient, respectively. Both leiomyosarcomas of gynaecological origin stained positive for OR/PR expression. One patient had previously undergone renal transplantation; this leiomyosarcoma was associated with EBV expression. Median survival was 56 months (range: 22–132 months) and eight, six and four patients remained alive at 1, 3 and 5 years, respectively. ConclusionsHepatic resection for leiomyosarcoma is associated with encouraging rates of 5-year overall and disease-free survival. The worse outcome that had been expected based on data derived from historical cohorts (partly comprising subjects with GIST) was not observed. An algorithm for pathological classification and treatment is suggested.

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.