Malignant fibrous histiocytoma - a case report

Fibrous histiocytoma is a mesenchymal neoplasm with benign and malignant varieties. This tumor mainly affects the skin of extremities in adults and may on rare occasions affect the oral cavity. The tumor has radiographic features in very rare cases. The present case report aims to conduct a clinicopathological-radiographic and immunohistochemical assessment and treatment of a patient with this lesion

Uterine tumor resembling ovarian sex cord tumor: A series of six cases displaying varied histopathological patterns and clinical profiles

Introduction: Uterine tumors resembling ovarian sex cord tumor (UTROSCT) are a unique group of neoplasms with diverse morphology and immunophenotypic characteristics, coexpressing sex cord, epithelial, and smooth-muscle markers. To date, less than 100 cases have been reported and there is paucity of data concerning their clinical behavior. Materials and Methods: All cases of uterine body tumors diagnosed over a period of two and a half years (2016-2018) were retrieved. Histopathological features were reviewed and extended panel of immunohistochemistry was performed to identify cases of UTROSCTs. Results: Six cases of UTROSCTs were identified with a median age of 46.5 years. Four of them presented with menorrhagia, while two with postmenopausal bleeding including one with a history of carcinoma breast. Three of these cases were initially misdiagnosed as endometrial stromal sarcoma and adenocarcinomas. They all underwent hysterectomy with bilateral salpingo-oophorectomy. Conclusion: It is considered a tumor with low malignant potential; however, one out of six cases (16.7%) in our study showed metastasis, within 1 year of diagnosis. It is important to recognize this entity as it mimics a wide range of both benign and malignant tumors. Molecular pathogenesis and exact management protocols remain elusive due to rarity,hence, multi-institutional studies are warranted.

Solitary fibrous tumor of the pleura mimicking pleural effusion

Solitary fibrous tumor of the pleura is a rare mesenchymal neoplasm of fibroblastic origin and represents less than 5% of primary tumors of the pleura. There are few reports in the literature about this disease, therefore the data on its presentation and management are derived from reports and retrospective analysis of case series from referral centers. In this report, we describe a case of a patient who presented this diagnosis during hospitalization, and following we briefly review the published literature of this pathology...

Cellular Plexiform Fibrohistiocytic Tumor / 대한피부과학회지

Plexiform fibrohistiocytic tumor (PFHT) is a rare mesenchymal neoplasm of intermediate malignancy and possibly of myofibroblast origin. It is morphologically divided into 3 groups: cellular, fibrous, and mixed. A 4-year-old girl presented with an irregular shaped subcutaneous mass on her left popliteal fossa for 6 months. The biopsy specimen showed multinodular tumor islands extending from dermis to subcutaneous layer, composed of histiocytes and osteoclast-like multinucleated giant cells, and circumscribed by fibrous tissue. Immunohistochemical staining was positive for CD68 in giant cells and histiocyte-like cells within tumor islands and faintly positive for smooth muscle actin around nodules. A cellular variant of PFHT was eventually diagnosed. Although PFHT comprises morphologically normal cells, it has the biological potential for malignant change and distal metastasis. Therefore, PFHT is categorized as a neoplasm of intermediate malignancy, and wide total excision with close follow-up is crucial.

Endoscopic Ultrasonography-guided Tru-Cut Biopsy for Differential Diagnosis in the Gastrointestinal Mesenchymal Neoplasm / 대한소화기내시경학회지

BACKGROUND/AIMS: Risk assessment by mitotic count in the gastrointestinal stromal tumor (GIST), the most common mesenchymal neoplasm, is important. EUS-guided fine- needle aspiration cannot obtain sufficient tissue for determination of mitotic count, but An EUS-guided Tru-Cut biopsy (EUS-TCB) can. The aim of this study was to evaluate clinical role of EUS-TCB in differential diagnosis of mesenchymal neoplasm and risk assessment of GIST. METHODS: We prospectively enrolled 18 patients who were diagnosed with submucosal hypoechoic tumors, 2~5 cm, in the muscular layer by EUS in Seoul National University Hospital from November, 2005, to July, 2007. The rates of adequate biopsy and possibility of risk assessment were evaluated. RESULTS: EUS-TCB was performed in 16 patients. The median tumor size was 2.7 cm, and adequate diagnosis was possible in 8 patients. Tumor size in the adequate specimen group was larger than in the inadequate group (p=0.021). The number of needle passes was higher in the adequate specimen group (p=0.012). Risk assessment by mitotic count was possible in only one case. CONCLUSIONS: EUS-TCB in the diagnosis and risk assessment of GIST was inadequate, in part because of insufficient operator experience and small tumors. Development of a new instrument is needed to obtain sufficient tissue.

Four Cases of Intrapulmonary Hamartoma: An ultrastructural study

Tumors of the lung and bronchi containing cartilage were known by a variety of names, chondroma, adenochondroma, chondromatous hamartoma and mixed tumor. This variation in nomenclatures explain the difference of illustration on the nature of these tumor. The concept pulmonary harmatomas are benign neoplasm and not developmental malformations, has gained wide acceptance in recent years. We have experienced four cases of intrapulmonary hamartoma which were all discovered during routine chest film check up for certificate of health and evaluation of other disease. One case is added further detailed histologic examination by electron microscopy. The age at time of the detection were 53 (male), 23 (male), 39 (female), and 56 (female) years old. The mean size is 4.3x3.7x3.4 cm. The locations were three left upper lobes and one right upper lobe. Lobectomy and wedge resecions were done. Cut surface showed promiment lobular structures, papillary configuration and multiple cleft like spaces. Predominant cellular components were cartilage but fat tissue in one of the four cases. Microscopic findings showed abundant hyaline cartilages bearing lobular configuration and overlying pseudostratified ciliated columnar and cuboidal epithelium. Fibromyxoid and undifferentiated cells were seen in myxoid and fatty tissue. Electron microscopic findings revealed stellate, undifferentiated mesenchymal cells bearing collagen formation, stellate smooth muscle and transition areas between undifferentiated mesenchymal cells and mature cartilage. Epithelial components were similar to terminal bronchiole and alveolar epithelium. These findings suggest the concept that intrapulmonary hamartoma represent a histologic specturm of benign mesenchymal neoplasms, which originate in peribronchial connective tissue.