Epithelioid Variant of High-Grade Myxofibrosarcoma in the False Vocal Fold: A Case Report

Myxofibrosarcoma (MFS) is a histologic subtype of malignant fibrous histiocytoma (MFH), with a predominant myxoid component. MFS is characterized by locally aggressive behavior and a high rate of local recurrence, however, with a good prognosis. Head and neck MFS accounts for 3% of all cases of MFS. To date, only two cases of laryngeal MFS have been reported. Owing to the rarity of MFS, the clinical characteristics and optimal treatment options remain controversial. Surgical resection with a clear margin is considered the treatment of choice. Compared to traditional MFS tumors, epithelioid variants have worse prognosis. Other factors associated with a poor prognosis of MFS tumors include inadequate surgical margins, large tumor size, old age, and high-grade tumors. Herein, we report a case of high-grade epithelioid variant MFS located in the false vocal fold, requiring total laryngectomy to obtain an adequate surgical margin. To our knowledge, this is the first case report of epithelioid variant of high-grade MFS presenting in the larynx.

Hepatic epithelioid angiomyolipoma: a case report

Angiomyolipoma (AML) is a rare benign solid tumor, of mesenchymal origin. Angiomyolipomas can be subdivided, according to their predominant component. The predominance of epithelioid cells characterizes the epithelioid variant (EAML). These express a more aggressive clinical behaviour with a greater potential for malignant transformation. In the absence of specific radiological features, the diagnosis of hepatic EAML depends on the pathological and immunohistochemical study. We present the case of an 80 years old female with a computed tomography (CT) scan showing a hepatic nodule at the transition of segments 5 and 8, and a left kidney nodule previously biopsied and confirmed to be a renal angiomyolipoma. A liver nodule biopsy was performed, whose histology revealed a probable gastrointestinal stromal tumor (GIST). The patient underwent a 5/8 subsegmentectomy and pathology report revealed epithelioid angiomyolipoma. After discussion of the case in the multidisciplinary tumor board, it was decided to perform a left nephrectomy, whose pathology revealed leiomyomatous angiomyolipoma. No further treatment and surveillance on outpatient clinic were decided. Hepatic EAML is a rare lesion that, although mostly benign, can exhibit malignant behavior with distant metastasis and local invasion. Its identification is of paramount importance. The definitive diagnosis is only possible through histological and immunohistochemical analysis. Additional studies are needed in order to establish diagnostic criteria and predictive characteristics of malignancy.

More than Pus – Primary Hepatic Epithelioid Angiomyolipoma Masquerading as Liver Abscess

Hepatic angiomyolipomas (AML) are rare mesenchymal tumours of which the epithelioid type is a rare type with malignant potential. We report a case of primary hepatic epithelioid angiomyolipoma masquerading as liver abscess. A 46-year-old man presented with a 5-day history of fever with epigastric pain and nausea. On the night of admission, his temperature spiked to 39°C, his blood pressure was 135/79, his heart rate 98, his liver function test revealed albumin 37 g/L, bilirubin 25 μmol/L, ALP 298 U/L, ALT 247 U/L, and AST 344 U/L. The clinical suspicion was hepatobiliary sepsis and intravenous ceftriaxone was commenced. CT of the abdomen showed an ill-defined hypodense focus in segment 4A/8 (4.5 × 3.5 cm) with a minimal fluid component implying a developing abscess or phlegmon. The images were reviewed by a radiologist and showed minimal fluid for percutaneous drainage. MRI of the liver was performed to further characterize the lesion and revealed a solid mass with nodular areas of arterial enhancement and washout, suspicious of neoplasm. Ultrasound-guided liver biopsy of the lesion was performed. Histology showed a histiocyte-rich epithelioid neoplasm consistent with the epithelioid variant of AML. Immunohistochemical staining was positive for human melanoma black 45, melan-A and cluster of differentiation 68. He successfully underwent liver resection of segment 4A/8 after 6 weeks of antibiotics. To our knowledge, this is the first reported case in the literature of primary hepatic epithelioid angiomyolipoma masquerading as liver abscess.

Impact of tumor histology and socioeconomic factors on survival of patients suffering from malignant vascular tumors of liver and hepatocellular carcinomas: A SEER database analysis.

e16612 Background: Primary malignant vascular tumors of the liver are rare, aggressive and poorly understood subtypes of liver cancers. In this analysis, we aim to determine the impact of tumor histology and other socio economic factors on survival of these tumors and hepatocellular carcinomas. Methods: Patients with malignant histopathological diagnoses of hepatocellular carcinoma not otherwise specified (HCC NOS), hepatocellular carcinoma with spindle cell (HCC SP), fibrolamellar (HCC F), clear cell (HCC CL), scirrhous (HCC SC) and pleomorphic variants ( HCC PL), combined hepatocellular carcinoma and cholangiocarcinoma (Mixed), hepatic angiosarcoma (AS), hemangioendothelioma (HE), its epithelioid variant (EHE) and hemangiopericytoma (HP) were identified using the SEER (version 2018) database. Overall survival was studied with Kaplan–Meier with the log rank method. Multivariable analysis was performed to assess the impact of race, ethnicity, marital and insurance status on survival of these patients. Results: We analyzed de-identified data of 104502 patients from the year 1973 to 2016 with hepatocellular carcinomas and malignant vascular tumors of liver (including 101851 patients with HCCNOS, 70 with HCC SP, 378 with HCC F, 104 with HCC SC, 593 with HCC CL, 23 with HCC PL, 950 with Mixed, 367 with AS, 36 with HE, 120 with EHE and 10 with HP ) respectively. Median overall survival (OS) of HCC NOS was calculated to be 7 months. Among hepatocellular carcinoma patients HCC F had the longest median OS of 29 months and HCC SP had the shortest median OS of 3months (P < 0.001). Additionally, among patients with malignant vascular tumors, AS had the shortest median OS (1 month) while patients with EHE had the longest median OS of 81 months (P < 0.001). Overall, married and insured patients had a better overall survival than unmarried and uninsured patients (P < 0.04). Conclusions: Malignant vascular tumors of liver are rare in comparison to hepatocellular carcinomas. Spindle cell variant of HCC and liver angiosarcomas carry the worst prognosis and fibrolamellar variant of HCC, hemangioendothelioma (HE) and its epithelioid variant (EHE) have the best prognosis. Insurance and marital status has a positive impact on overall survival of liver cancer patients.

Primary Cardiac Epithelioid Angiosarcoma in a Latin American Patient: Case Report and Literature Review

Cardiac angiosarcoma is a rare and clinically challenging pathology. It is a high-grade primary malignant tumor of the heart tissue that has many variants, of which the epithelioid variant is rarely present in the heart or the great vessels. As with many other cardiac tumors, it is mainly a diagnosis of exclusion and the initial diagnostic test is an echocardiogram followed by a biopsy with immunohistochemistry analysis to ascertain the type of tumor. The differential diagnosis of cardiac tumors is challenging due to the overlapping clinical manifestations with different cardiac tumors and systemic diseases. Cardiac angiosarcomas are often aggressive with a poor prognosis even with treatment. Herein, we present a case of the epithelioid variant of a cardiac angiosarcoma in addition to a thorough review of the recent literature on the clinical manifestation, diagnosis, and treatment of this type of tumors.