Adult-onset kaposiform hemangioendothelioma with neurofibromatosis type 1: A case report and literature review.

Kaposiform hemangioendothelioma (KHE) is a very rare, locally aggressive vascular neoplasm. It occurs mostly in children and is rarely observed in adults. It typically originates on the skin, later affecting the deep soft tissue of the extremities, head or neck, and retroperitoneum by infiltrative growth. It is locally aggressive, does not regress spontaneously, and tends to metastasize locally as well as to the regional lymph nodes. In this article, we report a case of adult-onset KHE with neurofibromatosis type 1. The patient presented to our department with a 2-month history of a painful ulceration in her left popliteal area. Since KHE had not previously been reported in patients with neurofibromatosis, the diagnosis was difficult due to the similarity of the skin manifestation to neurofibromatosis-associated lesions. We share our experience of diagnosing and treating this rare case of adult-onset KHE.

Solitary Piloleiomyoma in the Scalp.

Cutaneous leiomyomas can be classified into three types according to the site of origin: piloleiomyoma, angioleiomyoma, and dartoic (genital) leiomyoma. It might be expected that leiomyomas are commonly found on the scalp because there are many arrector pili muscles and vessels. However, leiomyomas are actually rarely reported in the scalp. Recently, we observed a case of cutaneous leiomyoma in the scalp and present our experience along with a literature review.

mRNA expression of CDH3, IGF2BP3, and BIRC5 in biliary brush cytology specimens is a useful adjunctive tool of cytology for the diagnosis of malignant biliary stricture.

Although advances have been made in diagnostic tools, the distinction between malignant and benign biliary strictures still remains challenging. Intraductal brush cytology is a convenient and safe method that is used for the diagnosis of biliary stricture, but, low sensitivity limits its usefulness. This study aimed to demonstrate the usefulness of mRNA expression levels of target genes in brush cytology specimens combined with cytology for the diagnosis of malignant biliary stricture. Immunohistochemistry for cadherin 3 (CDH3), p53, insulin-like growth factor II mRNA-binding protein 3 (IGF2BP3), homeobox B7 (HOXB7), and baculoviral inhibitor of apoptosis repeat containing 5 (BIRC5) was performed in 4 benign and 4 malignant bile duct tissues. Through endoscopic or interventional radiologic procedures, brush cytology specimens were prospectively obtained in 21 and 35 paitents with biliary strictures. In the brush cytology specimens, the mRNA expressions levels of 5 genes were determined by real-time polymerase chain reaction. Immunohistochemistry for CDH3, p53, IGF2BP3, HOXB7, and BIRC5 all showed positive staining in malignant tissues in contrast to benign tissues, which were negative. In the brush cytology specimens, the mRNA expression levels of CDH3, IGF2BP3, HOXB7, and BIRC5 were significantly higher in cases of malignant biliary stricture compared with cases of benign stricture (P = 0.006, P < 0.001, P < 0.001, and P = 0.001). The receiver-operating characteristic curves of these 4 mRNAs demonstrated that mRNA expression levels are useful for the prediction of malignant biliary stricture (P = 0.006, P < 0.001, P < 0.001, and P = 0.002). The sensitivity and specificity, respectively, for malignant biliary stricture were 57.1% and 100% for cytology, 57.1% and 64.3% for CDH3, 76.2% and 100% for IGF2BP3, 71.4% and 57.1% for HOXB7, and 76.2% and 64.3% for BIRC5. When cytology was combined with the mRNA levels of CDH3, IGF2BP3, or BIRC5, the sensitivity for malignant biliary stricture improved to 90.5%. The measurement of the mRNA expression levels of CDH3, IGF2BP3, and BIRC5 by real-time polymerase chain reaction combined with cytology was useful for the differentiation of malignant and benign biliary strictures in brush cytology specimens.

[A case of primary extragastrointestinal stromal tumor presenting as peritoneal dissemination].

Gastrointestinal stromal tumor (GIST) is the most common mesenchymal tumor of the gastrointestinal tract, but also occurs at a lower frequency in extra-gastrointestinal regions such as omentum, mesentery, retroperitoneum and undefined abdominal sites. This tumor is called extragastrointestinal stromal tumor (EGIST). EGIST is mostly diagnosed as a cystic mass, but rarely occurs as a disseminated abdominal tumor. We experienced a 70-year-old man with primary EGIST presenting as peritoneal dissemination. Abdominal CT showed diffuse peritoneal thickening with a large amount of ascites, but no definite mass lesion. Laparoscopic biopsy was performed and histologic findings showed tumor composed of epithelioid cells. In the results of immunohistochemical stains, the tumor showed positive reactivity with CD117 (c-kit), CD34, vimentin and actin, but negative reactivity with desmin and S-100 protein. On account of unresectability and histologic parameters of malignant behavior, he was started on imatinib.

Ileal malignant melanoma presenting as a mass with aneurysmal dilatation: a case report.

Malignant melanoma is the most common metastatic tumor of the gastrointestinal tract and can present with fairly common constitutional symptoms. A 36-yr-old woman was found to have a secondary malignant melanoma in the terminal ileum with profuse aneurysmal dilatation, which is not the typical presentation of the malignant melanoma in the small intestine. Radiologic studies revealed a large tumor involving the distal ileum with aneurysmal dilatations having afferent and efferent loops, which needed to be differentiated from malignant lymphoma and other gastrointestinal tumors. Exploratory laparotomy was done, and we found a huge mass with plentiful aneurysmal dilatations; much the same of the findings from the previous studies. Segmental resection with the surrounding omentum was done followed by end-to-end anastomosis between both ends of the remaining ileum. She had been free from any evidence of the local or systemic recurrence for one year after the completion of eighteen months of the subcutaneous interferon treatment; postoperatively however, the occurrence of metastatic mass at the right axilla rendered us from complete resection due to severe penetration into the vital nerves and vessels in the axilla.