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Background/Aim: Epithelioid hemangioendothelioma (EHE) of the liver is an uncommon vascular tumor with variable clinical courses ranging from stable disease to fatal outcome. EHE can mimic epithelioid angiosarcoma, which has a more aggressive behavior, especially in a small biopsy sample. EHEs are known to have the WWTR1-CAMTA1 fusion gene, and nuclear expression of CAMTA1 by immunohistochemistry (IHC) has been reported in about 90% of EHEs in multiple organs. Our study aimed to validate the diagnostic utility of CAMTA1 expression in EHEs, especially in the liver. Patients and Methods: IHC was performed using anti-CAMTA1 antibody in 34 tumors (24 hepatic EHEs and 10 angiosarcomas). In CAMTA1-negative EHEs, TFE3 IHC was performed. Results: Of the 24 hepatic EHEs, 22 (91.6%) showed nuclear staining for CAMTA1. One of two CAMTA1negative cases showed TFE3 positivity. The other case was negative for TFE3. Meanwhile, all 10 angiosarcoma cases had no CAMTA1 expression. Conclusion: CAMTA1 is a highly sensitive and specific marker for diagnosis of hepatic EHE. It is helpful for differentiation of hepatic EHE and angiosarcoma, especially in small biopsy samples.

Breast mucinous carcinoma (MC) is defined as floating tumor clusters within extracellular mucin pools [1]. MC is divided into two subtypes, pure and mixed, according to the proportion of mucinous component in the tumor [1]. Pure MC (PMC) is composed of more than 90% mucinous component, is a rare type of breast cancer accounting for approximately 2% of invasive cancers, and usually occurs in elderly patients (> 55 years) [1,2]. PMC generally has a low nuclear grade and good prognosis [1,3]. Typical PMC is positive for estrogen receptor (ER) and progesterone receptor (PR), whereas it is negative for human epidermal growth factor receptor 2 (HER2) [1,3,4]. While HER2 overexpression occurs in 15%-20% of invasive breast cancers [5], it has been reported in only 2.6-9.0% of PMC [4,6-8]. Clinicopathologic characteristics of HER2-positive PMC have not been studied well because of the rarity of this entity. Herein, we investigate the clinicopathologic characteristics of HER2positive PMC.

Purpose: Salivary gland cancer (SGC) in the oral cavity is not common and has been less studied in comparison with oral squamous cell carcinoma (SCC). This study aimed to identify the clinical characteristics and outcomes of SGC in the oral cavity compared with oral SCC. Methods: The medical charts of the patients with SGC (N = 68) arising from minor salivary glands and SCC (N = 750) in the oral cavity between 1995 and 2017 were reviewed retrospectively. The clinical and pathological factors and treatment outcomes were compared to identify clinical differences between oral SGC and SCC in total cases and in tumor size and subsite (propensity score)-matched pairs (N = 68 in each group). In addition, pattern of local invasion was pathologically assessed in a subset of SGC and SCC tumors. Results: Patients with SGC in the oral cavity showed >90% survival at 5 years. Most common pathologies of SGC were mucoepidermoid carcinoma (39.7%) and adenoid cystic carcinoma (35.3%), where high-grade tumors (including adenoid cystic carcinomas having solid components, grade 2 or 3) represented only 36.8%. Compared with oral SCC, surgery for SGC had narrow surgical safety margin. However, local control was very successful in SGC even with <5 mm or positive resection margin through surgery plus adjuvant radiation treatments or surgery alone for small low-grade tumors. Pathologic analysis revealed that the frequency of oral SGC with infiltrative tumor border was significantly lower than that of oral SCC (46.4 vs. 87.2%, P < 0.001). Conclusions: SGC in the oral cavity represents relatively good prognosis and has a locally less aggressive pathology compared with oral SCC. Adjuvant radiation can be very effective to control minimal residual disease in oral SGC. Our study proposed that a different treatment strategy for oral SGC would be reasonable in comparison with oral SCC.

Background/Aim: Primary hepatic angiosarcoma (PHA) is a rare disease entity with variable morphologic features. Recent findings regarding ROS1 gene rearrangements in PHA may lead to new targeted therapies. Patients and Methods: Thirteen cases (4 resected specimens and 9 biopsy samples) underwent histologic review and morphologic patterns were classified according to a previous study as 1) sinusoidal, 2) peliotic, 3) vasoformative, and 4) solid (epithelioid/spindled). ROS1 immunohistochemistry and investigation of the presence of a ROS1 fusion gene by reverse transcription-polymerase chain reaction were performed in available cases. Results: Eight of 13 cases (62%) showed vasoformative patterns. Three cases (23%) were classified as sinusoidal and two (15%) as solid patterns. Mortality rate was 90% (9/10) except for three patients lost in follow up. Only one patient is still alive and has survived for 8 months with the disease. All cases tested did not have ROS1 expression (0/9) or a ROS1 fusion gene (0/4). Conclusion: We report 13 cases of PHA with 90% mortality. Vasoformative PHA is the most common histologic type. New findings on ROS1 fusion gene rearrangements could lead to the development of novel targeted therapeutics for PHA patients with dismal prognosis.

Mucinous carcinoma (MC) is a rare subtype of breast cancer, which is composed of tumor cells floating in the abundant extracellular mucin. This form of cancer is usually estrogen receptor/progesterone receptor positive and human epidermal growth factor receptor 2 (HER2) negative. Here, we present a case of HER2-positive MC with an unusual signet ring cell differentiation. It is very rare that a breast tumor consists entirely of signet ring cells. The tumor showed pathologic complete response (pCR) after neoadjuvant chemotherapy with trastuzumab and pertuzumab. pCR of HER2-positive MC has rarely been described in literature. It is important to consider the biological heterogeneity of MCs for effective management.

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