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.

Mikulicz's Disease with Progressively Transformed Germinal Centers-type Immunoglobulin G4-related Lymphadenopathy Mimicking Sjogren's Syndrome

Immunoglobulin G4-related disease (IgG4-RD) is a systemic disease, and lymphadenopathy is frequently observed in these patients. Among the 5 subtypes of IgG4-related lymphadenopathy, progressively transformed germinal centers (PTGC)-type IgG4-related lymphadenopathy possesses a unique characteristic that differentiates it from the other 4 subtypes. Here, we report on a rare case of PTGC-type IgG4-related lymphadenopathy accompanying Mikulicz's disease. A 39-year-old female complained of a left cervical mass and bilateral upper eyelid hypertrophy. The serum level of IgG4 was elevated, and computed tomography showed enlargement of the bilateral lacrimal and submandibular glands and left cervical lymph node. Excisional biopsy of a submandibular gland and cervical lymph node was performed, and the histopathologic findings revealed Mikulicz's disease accompanied by PTGC-type IgG4-related lymphadenopathy. After treatment of the patient with oral prednisolone and azathioprine, the patient's appearance improved. To the best of our knowledge, no case of PTGC-type IgG4-related lymphadenopathy has been previously reported in Korea.

A Case of Primary Extragastrointestinal Stromal Tumor Presenting as Peritoneal Dissemination / 대한소화기학회지

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.

Undifferentiated Adenocarcinoma of the Colon with Rhabdoid Features / 대한소화기내시경학회지

Malignant rhabdoid tumors were first described in 1978 by Beckwith and Palmer as a rare variant of Wilms' tumors with a "rhabdomyosarcomatoid" pattern and a particularly poor prognosis. Week reclassified this disease as a distinct disease in 1989 and thereafter, there have been several reports about malignant rhabdoid tumor that occurred in various organs, including the colon. The histologic characteristics of rhabdoid tumor are noncohesive or loosely cohesive cells with high cellularity, an eccentric large nucleus and eosinophilic cytoplasm, and the cytoplasm is usually positive for vimentin and it contain hyaline inclusions. On immunohistochemical staining, the cells are usually positive for vimentin and cytokeratin and they are negative for desmin. This tumor progresses rapidly and it has a very poor prognosis, but survival is better if there is no lymphatic or distant metastasis. We experienced a patient who suffered with undifferentiated adenocarcinoma with rhabdoid features in the ascending colon.

Metastasis of adenoid cystic carcinoma of the minor salivary grand to the scalp : A case report

Xanthogranulomatous Inflammation Presenting as a Submucosal Mass of the Stomach / 대한소화기내시경학회지

Xanthogranulomatous inflammation is a rare chronic inflammatory condition that is characterized by aggregation of lipid-laden foamy macrophages (xanthoma cells). Although the precise pathogenesis of xanthogranulomatous inflammation is not well understood, various mechanisms have been proposed, including chronic recurrent infection, the presence of gallstones, immunologic disorders and defective lipid transport. This disease entity is well recognized in the kidney and gallbladder, yet involvement of the gastrointestinal tract is extremely rare and the involvement of both the stomach and colon has never been reported on. A coexisting malignancy rarely has been reported in a patient with xanthogranulomatous inflammation. This might present as an inflammatory mass-like lesion with infiltration to the surrounding tissues, and so this often mimics advanced cancer. Therefore, a surgical operation together with careful pathological evaluation is required for making the precise diagnosis. We herein report on a case of xanthogranulomatous inflammation that presented as a submucosal mass in the stomach which was a huge irregular mass involving transverse colon.

A Case of Esophageal Gastrointestinal Stromal Tumor Treated by Endoscopic Submucosal Dissection following an Initial Mucosectomy Using a Transparent Cap / 대한소화기내시경학회지

Gastrointestinal stromal tumor (GIST) is the most common mesenchymal neoplasm of the gastrointestinal (GI) tract, but the lesion occurs rarely in the esophagus. Although only 10~30% of GISTs are malignant at the time of diagnosis, many reports show that it is difficult to predict the prognosis and underlying potential of non-malignant ones. Thus, the surgical removal of GISTs is even being proposed as a standard treatment. Recently the endoscopic removal of submucosal tumors of the GI tract is being done in popular, but it has been still considered as both difficult and risky to resect a tumor originating from the muscularis propria layer using the endoscopic methods. Herein, we report a case of an incidentally found submucosal tumor originating from the muscularis propria treated with an endoscopic submucosal dissection method which was performed after a preceding mucosectomy using a transparent cap. The mass turned out to be an esophageal GIST.

A case of multiple gastric carcinoid tumors associated with Sjogren's syndrome / 대한내과학회지

Carcinoid tumors are rare endocrine neoplasms arising from the enterochromaffin or enterochromaffin-like cells. Gastric carcinoids associated with autoimmune atrophic gastritis and hypergastrinemia, are usually multiple and the prognosis are better compared to solotary lesions with out hypergastrinemia. Gastric carcinoids are commonly associated with other endocrine disorders or tumors, but any associations with autoimmune disorders other than autoimmune atrophic gastritis have rarely been reported. Sjogren's syndrome is an autoimmune exocrinopathy that primarily affects the salivary glands, but it can also involve almost any other part of the gut. The most common form of gastrointestinal involvement in Sjogren's syndrome is chronic atrophic gastritis, which can lead to hypergastrinemia and the subsequent development of carcinoid. However, gastric carcinoid tumor associated with Sjogren's syndrome has not yet been reported on. To the best of our knowledge, this is the first such case in the world. We report on this case along with review of the related literature.

A Case of Avascular Necrosis of Mandible Associated with the Use of Bisphosphonate in a Patient with Rheumatoid Arthritis and Osteoporosis / 대한류마티스학회지

Bisphosphonates decrease osteoclastic activity and reduce bone resorption, therefore they are used in the treatment of patients with hypercalcemia secondary to malignancy or metastatic bone cancer, Paget's disease and osteoporosis. Recently, cases of jaw bone necrosis have been reported to be associated with the use of bisphosphonates. In patients with rheumatoid arthritis, osteoporosis is frequently developed because of osteoclast activation and steroids which are used for treatment and bisphosphonates are frequently prescribed. A 76-year-old female patient with rheumatoid arthritis was treated with alendronate for the management of osteoporosis for 36 months, and she was presented with pain and swelling on left mandibular area and gingival pus discharge. The patient was treated with curettage of necrotic bone, and biopsy showed avascular necrosis of mandible.

Basaloid Squamous Cell Carcinoma of the Rectum Manifesting as Multiple Submucosal Lesions / 대한소화기내시경학회지

Basaloid squamous carcinoma is a rare, pooly differentiated variant of squamous cell carcinoma. It occurs in various sites, including the upper digestive tract, the esophagus, lung, anus, cervix and thymus. It has been postulated that basaloid carcinoma may arise from outside of the anal canal, such as at where the cloacogenic embryologic cells rest, the squamous metaplastic epithelium, or the totipotential basal cells. This tumor is a distinct entity that should be carefully distinguished from basal cell carcinomas of the anal canal, which is a condition that has a very good prognosis, and anal or perianal squamous cell carcinomas, which have a different path of spread and they vary considerably in their behavior. We report here on a patient with basaloid squamous carcinoma in the distal rectum that manifested as multiple submucosal lesions, and the patient presented with abdominal pain and blood tinged stool.

Intrasellar Schwannoma: A Case Report

Schwannomas usually arise from sensory nerves, and most often from the vestibular component of the acoustic nerve. Intrasellar and parasellar schwannomas are exceedingly rare. It is difficult to distinguish them from typical pituitary macroadenomas because of their clinical and radiological resemblance. In this report, we present an unusual case of an intrasellar schwannoma with a suprasellar extension that radiographically simulated a pituitary macroadenoma.

Expression of estrogen receptor beta, estrogen receptor alpha and cyclooxygenase II in advanced breast cancer / 한국유방암학회지

PURPOSE: Although the role of the estrogen receptor alpha (ER alpha, previously called the estrogen receptor) in breast cancer is well established, that of the second human estrogen receptor (ER), estrogen receptor beta (ER beta), remains uncertain. The expression of cyclooxygenase II (COX II) could also be regulated by sex steroids such as estrogen and progesterone. To investigate whether the expressions of the ER beta, ER alpha, and COX II are elevated in more aggressive breast cancers, the expression of the ER beta was studied by immunohistochemical staining in 20 primary breast cancer and original breast cancer tissues from 20 recurrent cancer patients, and its associations with ER alpha and cyclooxygenase (COX) II were evaluated. METHODS: Paraffin tissue sections from 40 breast cancers, surgically excised at the Department of Surgery, the Catholic University of Korea. were obtained. The immunohistochemical analysis was conducted on 20 non-recurrent, and 20 recurrent primary breast cancer tissues, using polyclonal antibodies to ER beta, ER alpha, and the corresponding monoclonal antibodies to COX II. RESULTS: Of the 40 patients, 15 (37.5%) were ER beta-positive, 30 (75%) were ER alpha-positive, and 24 (60%) were COX II-positive. The ER bata status was not related to the tumor size or menopausal status, but was related to the nodal status. The stati of ER alpha and COX II were not related to other clinico-pathological factors. The ER beta positivity was significantly more frequent in the study than the control group. (ER beta, p = 0.0222; ER alpha p = 0.1441; COX II, p = 1.00) The presence of ER beta was significantly related to the expression of ER alpha and COX II (p = 0.0455, p = 0.0381, respectively). CONCLUSION: These results suggest that the expression of ER beta is associated with early recurrence in breast cancer and the expression of COX II in the presence of ER beta implies the possibility of prognostic significance.

Expression of estrogen receptor beta, estrogen receptor alpha and cyclooxygenase II in advanced breast cancer / 한국유방암학회지

PURPOSE: Although the role of the estrogen receptor alpha (ER alpha, previously called the estrogen receptor) in breast cancer is well established, that of the second human estrogen receptor (ER), estrogen receptor beta (ER beta), remains uncertain. The expression of cyclooxygenase II (COX II) could also be regulated by sex steroids such as estrogen and progesterone. To investigate whether the expressions of the ER beta, ER alpha, and COX II are elevated in more aggressive breast cancers, the expression of the ER beta was studied by immunohistochemical staining in 20 primary breast cancer and original breast cancer tissues from 20 recurrent cancer patients, and its associations with ER alpha and cyclooxygenase (COX) II were evaluated. METHODS: Paraffin tissue sections from 40 breast cancers, surgically excised at the Department of Surgery, the Catholic University of Korea. were obtained. The immunohistochemical analysis was conducted on 20 non-recurrent, and 20 recurrent primary breast cancer tissues, using polyclonal antibodies to ER beta, ER alpha, and the corresponding monoclonal antibodies to COX II. RESULTS: Of the 40 patients, 15 (37.5%) were ER beta-positive, 30 (75%) were ER alpha-positive, and 24 (60%) were COX II-positive. The ER bata status was not related to the tumor size or menopausal status, but was related to the nodal status. The stati of ER alpha and COX II were not related to other clinico-pathological factors. The ER beta positivity was significantly more frequent in the study than the control group. (ER beta, p = 0.0222; ER alpha p = 0.1441; COX II, p = 1.00) The presence of ER beta was significantly related to the expression of ER alpha and COX II (p = 0.0455, p = 0.0381, respectively). CONCLUSION: These results suggest that the expression of ER beta is associated with early recurrence in breast cancer and the expression of COX II in the presence of ER beta implies the possibility of prognostic significance.

Superficial Thrombophlebitis of the Dorsal Vein of the Penis (Penile Mondor's Disease) / 대한비뇨기과학회지

Herein, two cases of penile Mondor's disease are described. The first was a 45-year-old man who complained of a small subcutaneous induration, with localized discomfort at the distal rim of the coronal sulcus of the penis. The other was a 46-year-old man who complained of similar symptoms of penile induration. The lesions were surgically removed under local anesthesia, and found to be thromboses of the penile superficial dorsal vein. As a result, the diagnoses of Mondor's disease of the penis were confirmed.

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.

Three Cases of Biliary Cystadenoma and Biliary Cystadenocarcinoma

A biliary cystadenoma and a cystadenocarcinoma are rare intrahepatic cystic neoplasm. The clinical feature is not marked but abdominal fullness and mass are the most common symptoms. The tumor is commonly a large multilocular cystic mass which requires hepatectomy for cure. We experienced one case of biliary cystadenoma and two cases of biliary cystadenocarcinoma. The biliary cystadenoma case was a 58-year-old female with right upper quadrant discomfort for 5 months and a 17 cm sized multilocular cystic mass. The serum CA 125 level was elevated but returned to normal level after resection. A right hepatectomy was performed and the patient has had no recurrence for 14 months after the resection. One of the biliary cystadenocarcinoma cases was a 42-year-old man with a 12 cm sized multilocular cystic mass in the right upper quadrant of his abdomen. A right hepatectomy was performed and the patient has had no recurrence for 12 months after the resection. The other biliary cystadenocarcinoma case was a 70-year-old man with right upper quadrant pain and a 5 cm sized cystic mass. A left hepatecomy was performed and the patient has had no recurrence for 8 month after the resection. The treatment of choice for a biliary cystadenoma or cystadenocarcinoma is complete resection. We report three cases of biliary cystadenoma and cystadenocarcinoma with a review of the literature.

A Case of Arteriovenous Hemangioma of the Scrotum and the Urethra / 대한비뇨기과학회지

Hemangioma is the most common congenital anomaly in the human. It represents a proliferation of endothelial cells and has been characterized as a benign neoplasm. Hemangiomas involving the genitalia comprise 2% of all such masses. Arteriovenous hemangioma is a benign congenital lesion that rarely involves the scrotum or urethra and primarily presents during childhood. We report a case of subcutaneous scrotal and urethral hemangioma that has caused persistent urethral bleeding in a 14-year-old boy who was treated by en bloc excision.

Transcervical expulsion of a submucosal myoma as a result of uterine artery embolization / 대한산부인과학회잡지

Uterine artery embolization was introduced to arrest post-partum hemorrhage 20 years ago. It has also been used to control severe hemorrhage from uterine gestational trophoblast tumors, carcinoma of the uterus, uterine arteriovenous malformations, and cases of pelvic trauma. More recently, transcatheter uterine artery embolization is a new treatment for uterine leiomyoma. A 33-year old married woman with 2 children had a history of heavy pelvic pain and pressure. Ultrasound and MRI investigation showed a bulky 12 x 10cm submucosal myoma in right lower uterine segment. The both uterine artery embolization was performed via a bilateral femoral artery. We present a case in which successful embolization of the uterine arteries in a woman with submucosal myoma resulted in a subsequent transcervical expulsion of large pieces of the dominant fibroid after 3 month of treatment with a brief review.

The Expression of p53, c-erbB-2 and nm23 Proteins in Breast Cancer

Recently, p53, c-erbB-2 and nm23 proteins have been studied in breast cancer. The expression of p53 protein indicates the mutation of p53 gene known as a tumor supressor gene, and c-erbB-2 gene amplification has been considered an indicator of poor prognosis and nm23 a metastsis suppressor gene. In order to elucidate the roles and relations of these proteins in the develpoment, progression and metastasis in breast cancer, we studied 89 cases of invasive breast cancer and 32 cases of lymph node metastasis for the expression of p53, c-erbB-2 and nm23 proteins using an immunohistochemical method. The results were as follows: 1) The expression rates of p53, c-erbB-2, and nm23 proteins in breast cancer were 40.4%, 34.8% and 55.1%, respectively. Co-expression of p53 protein and c-erbB-2 protein was found in 20.2% of cases, showing the highest incidence in poorly differentiated type (40%). 2) p53 protein expression was increased in poorly differentiated type but was not statistically significant. On the other hand, the expression of nm23 protein was decreased in poorly differentiated type, which was statistically significant (p<0.05). 3) The correlation of p53 protein expression with c-erbB-2 protein expression was statistically significant (p<0.05) but that with nm23 protein was not. 4) In the cases with lymph node metastasis, discordant expression of p53, c-erbB-2 and nm23 proteins between primary tumor and the lymph node metastatic tumor was found in 9.4%, 3.1% and 18.8% of cases, respectively. The above results suggest that overexpression of p53 and c-erbB-2 proteins and downregulation of nm23 protein are associated with the tumor progression in the breast cancer.