Type and Incidence of Soft Tissue Sarcomas in Korea: 2001-2007

BACKGROUND: The Korean Bone and Soft Tissue Pathology Study Group of the Korean Society of Pathologists conducted a nationwide retrospective analysis of soft tissue sarcoma (STS) to provide the clinicopathologic characteristics of STS within the population of the Republic of Korea. METHODS: The cases of STS were collected during a 7-year period (2001-2007) from 19 institutes in Korea. All cases were classified according to the histologic criteria proposed by the World Health Organization. Clinicopathologic data were reviewed. RESULTS: Data from 722 patients (median age, 50 years) were collected. Data showed a slight male predominance. The most frequent types of STS in decreasing order were liposarcoma, malignant fibrous histiocytoma, leiomyosarcoma, and synovial sarcoma. STS occurred throughout the body, although approximately half (47.8%) were located in the extremities. The majority of STS was histologically classified as high grade with a large tumor size (>5 cm). The overall survival rate for the patients was 76.3% (median follow-up time, 26 months; range, 1 to 89 months). Histologic grade, tumor size, American Joint Committee on Cancer stage, tumor site, and resection status were prognostic. Significant independent adverse prognostic factors were large tumor size (>5 cm) and tumor site other than extremities. CONCLUSIONS: We reported the distribution and characteristics of STS in the Republic of Korea.

Fine Needle Aspiration Biopsy of a Myxoid Leiomyosarcoma with Epithelioid Features and It Metastasized to the Abdominal Wall: A Case Report

We present the cytologic findings observed in a fine needle aspiration biopsy specimen of a rare myxoid variant of leiomyosarcoma with epithelioid features and the tumor had metastasized to the abdominal wall. The aspirate showed hypercellularity in a hemorrhagic background. Some large 3-dimensional aggregates of spindle cells were observed. Each cell had a solitary ovoid-to-elongated nucleus with finely granulated chromatin, one or two small distinct nucleoli and an irregular nuclear membrane. There were irregular fascicles of spindle cells with cigar-shaped, blunt-ended nuclei admixed with inflammatory cells. Epithelioid cells with a rather narrow, dense cytoplasmic rim and a well-defined cell border were embedded in a myxoid matrix in a cord-like and cluster arrangement. The matrix appeared as a pale green substance with sharply defined edges. There were very few mitoses. These cytologic features were the same as those of a uterine myxoid leiomyosarcoma that was surgically excised 7 years ago, and immunohistochemical staining revealed the smooth muscle origin of the tumor.

Loss of Heterozygosity on Chromosome 15q15 Near Thrombospondin-1 Gene in Breast Carcinomas

BACKGROUND: Chromosome 15q15 near the thrombospondin-1 (THBS-1) gene may be associated with tumor progression and metastasis. To clarify the potential role of the15q15 region in progression of breast carcinoma, we investigated the loss of heterozygosity (LOH) and the microsatellite instability (MSI) status of chromosome 15q15. Methods : LOH and MSI were detected in 84 breast carcinoma specimens using PCR-based microsatellite analysis with three microsatellite markers. METHODS: LOH and MSI were detected in 84 breast carcinoma specimens using PCR-based microsatellite analysis with three microsatellite markers. RESULTS: Of 77 breast carcinomas containing the heterozygous alleles, 25 (32%) showed LOH in at least one microsatellite marker. Partial LOH and total LOH were detected in 14 (18.27%) and 11 (14.3%) cases. The total LOH were inversely correlated with node metastasis. A single LOH at D15S514 was inversely correlated with nuclear grade and a single LOH at the D15S129 allele was associated with increased expression of the THBS-1 gene. MSI-positive breast carcinomas detected in 14 (17%) cases showed no correlation with any clinicopathologic feature. CONCLUSIONS: These results indicate that loss of the chromosome 15q15 region delays the progression of breast carcinoma because the magnitude of LOH is large and involves the THBS-1 gene and additional genetic elements. The genes located on chromosome 15q15 probably play a tissue-type-dependent role in malignant growth of the tumor.

Pathological Findings of Crohn's Disease in the Stomach

BACKGROUND: The incidence of Crohn's disease in the upper digestive tract, and especially in the stomach, is recently increasing. Focal inflammatory reaction without Helicobacter pylori (H. pylori) infection is thought to be the characteristic pathologic findings suggesting Crohn's disease in the stomach. Yet gastric involvement of Crohn's disease has not been studied in Korea. We studied the endoscopic and pathologic findings of patients with Crohn's disease in the stomach by taking biopsies. METHODS: Thirty patients with Crohn's disease who underwent gastroduodenoscopy followed by biopsies were included in the study. The pathology of the gastric biopsy specimens and the presence of H. pylori were evaluated. RESULTS: Among 30 cases, 22 cases (73.3%) were H. pylori negative and 8 cases (26.7%) were H. pylori positive. For the H. pylori negative cases, all but one cases showed pit abscess and focal lymphocytic collections in the antrum. Granulomas were found in 6 cases (20%) and they were exclusively located in the antrum. CONCLUSIONS: In the stomach, pit abscess and focal lymphocytic collections that are not associated with H. pylori infection are the characteristic pathologic findings found in Crohn's disease.

Xanthomatous Pseudotumor of the Breast: A Brief Case Report

Inflammatory (xanthomatous) pseudotumors of the breast are very rare and this case is the first reported case in Korea. A healthy, pregnant 29-year-old woman presented with a right breast mass. Ultrasonography of the breast revealed a 1.8 x 1.9 x 1.1 cm, sized lobulated, partially spiculated mass in the upper and outer quadrant of the breast. Macroscopically, the mass was well circumscribed, bright yellow, and lobulated. Microscopically, the tumor was composed of foamy histiocytes with multifocal neutrophilic infiltration, accompanying chronic inflammatory cellul infiltration, fibrosis and ductal-lobular atrophy.

Aspergillous Hypophysitis: A Case Report

Aspergillous hypophysitis is an unusual cause of sella turcica enlargement, and this malady has a clinical presentation as a pituitary tumor, and especially as a pituitary adenoma. We report here on a case of aspergillous hypophysitis that developed in a 37-year-old healthy woman. She first experienced a blurred vision with amenorrhea and galactorrhea. Three months later, the CT scan revealed an intrasellar mass. The patient underwent a transsphenoidal exploration of the sella turcica for a presumed pituitary tumor. Histologically, the pituitary displayed necrotizing granuloma with the acutely branching fungal hyphae of Aspergillus.

The Expression of Matrix Metalloproteinase-9 and Tumor Angiogenesis in Human Osteosarcoma

BACKGROUND: Matrix metalloproteinase-9 (MMP-9) is a matrix-degrading enzyme that's believed to play a crucial role not only for tumor invasion and metastasis, but also for a variety of stromal reactions, including neovascularization. The aim of this study was to investigate the expression of MMP-9 and to compare its expression with the angiogenesis activity in human osteosarcoma. METHODS: Archival tumor tissue samples from 20 patients with osteosarcoma were analyzed by performing immunohistochemistry for the expression of MMP-9 and CD34. The vascularity was measured as the average microvascular density (MVD) of the CD34-positive vessels. The clinical information was obtained through searching the computerized retrospective database from the tumor registry. RESULTS: MMP-9 was expressed in 90% (18/20) of the tumors we examined. The MVD ranged from 10.5 to 179.7 with a mean of 64.9. There was no significant correlation between the MMP-9 expression and the MVD (p=.613). The MMP-9 expression was not associated with any of the clinicopathologic variables, whereas the MVD showed an increasing tendency according to the metastasis status (p=.073). CONCLUSIONS: We demonstrated that MMP-9 activation is likely to occur in human osteosarcoma. However, there was no direct involvement of MMP-9 with tumor angiogenesis. It is noteworthy that MVD may aid physicians to predict the presence of distant metastasis in osteosarcoma patients.

Hyalinizing Spindle Cell Tumor with Giant Rosettes with Pulmonary Metastasis After a Long Hiatus: A Case Report

"Hyalinizing spindle cell tumor with giant rosettes" (HSCTGR) is a recently described tumor, which is regarded as an unusual variant of low-grade fibromyxoid sarcoma. Proof of a metastatic potential was lacking. The patient in the report was a 35-yr-old woman who showed multiple bilateral pulmonary nodules with massive pleural effusion in the right side. She had a history of a mass excision in the right thigh 11 yrs ago at another hospital, which was reported as a "leiomyoma". Two years before this presentation, the patient received a routine chest radiograph which demonstrated bilateral multiple pulmonary nodules. A lobectomy of the left upper lung was performed. The histological findings revealed a well-circumscribed nodule that was characterized by a spindle-shaped fibrous to hyalinized stroma with criss-crossing short fascicles and giant collagen rosettes surrounded by a rim of spindle-shaped cells. Electron microscopy confirmed the fibroblastic nature of the tumor. This case, in addition to at least two other cases reported in the literature, demonstrates that the HSCTGR is a malignant neoplasm with the capacity to metastasize after a long hiatus.

HER-2/neu Oncogene Amplification by Chromogenic in situ Hybridization in 130 Breast Cancers Using Tissue Microarray and Clinical Follow-up Studies

Determining of HER-2/neu oncogene amplification has become clinically important for managing breast cancer. Fluorescent in situ hybridization (FISH) and immunohistochemistry (IHC) are currently regarded as the standard methods. Chromogenic in situ hybridization (CISH) was investigated as a new modification with an accurate, sensitive technique. From 1998 to 2002, using CISH and IHC, the amplification and protein expression of the HER-2/neu oncogene were examined using paraffin sections in 130 breast carcinomas and to determine the prognostic role of HER-2/neu for outcome after a follow-up of 24- 64 months. Amplifications by CISH and overexpression by IHC were observed in 28 (22%) and 27 cases (20.8%), respectively. Of the 104 patients, 20 patients (19.2%) with amplification had a shorter disease-free interval (34.9 months vs. 38.0 months in controls) (p=0.372). 15 patients (14.4%) had a disease recurrence, but there is no significant difference between 3 patients amplifying the oncogene and 12 patients without oncogene (20.6 months vs. 19.6 months) (p=0.862). 6 patients (5.8%) of these died. CISH is a useful alternative, particularly for confirming the IHC results. There is no relationship between the early recurrence and the HER-2/neu positive group, but lymph node status was statistically significant.

Cytology of the Uterine Cervico-vaginal Smear of Clear Cell Adenocarcinoma in Uterine Cervix: Report of a Case / 대한세포병리학회지

Primary clear cell adenocarcinoma of uterine cervix is rare and cytomorphology in the vaginal smear have not been previously described in Korean literatures. The cytologic characteristics of clear cell adenocarcinoma of the uterine cervix include : malignant cells with abundant, finely vacuolated cytoplasm ; hobnail appearance ; and distinctive basement membrane-like hyaline materials within cellular aggregates. A 36-year-old woman presented with vaginal bleeding. Cytologic examination of vaginal smear and histopathologic examination of a radical hysterectomy specimen allowed the diagnosis of hemorrhagic tumor in the uterine cervix as a clear cell adenocarcinoma. Cytologic findings were very characteristic. The tumor cells had abundant, pale, finely vacuolated cytoplasm with indistinct cytoplasmic membrane. The nuclei were round to oval with finely dispersed chromatin. Extracellular basement membrane-like hyaline substance, which stained a light green color in Papanicolaou's preparation, was frequently observed within the cancer cell clusters.

Fine Needle Aspiration Cytology of the Branchiogenic Carcinoma: Report of A Case / 대한세포병리학회지

Development of an invasive squamous cell carcinoma within a branchial cleft cyst (branchiogenic carcinoma) is very rare and this case is the first description of cytologic findings in Korea. A 62-year-old man presented with a 5-month history of an enlarged right neck mass. Fine needle aspiration cytology of the mass showed low cellularity and the background containing proteinaceous debris, inflammatory cells, and histiocytes suggested a cystic nature. Abundant lymphocytes, a few anucleated squames, and rare atypical squamous cells with pyknotic nuclei and abnormal keratinization were also noted. After excision of the mass, histologic findings were consistent with a branchiogenic carcinoma. We recommend to raise the possibility of carcinoma (primary or metastatic) when we observe a few dyskeratotic cells in a cystic mass within the neck area, especially in an old male.

Primary Adenocarcinoma of the Seminal Vesicle: A Case Report

Primary adenocarcinoma of the seminal vesicle is an extremely rare tumor, and its prognosis has been known to be poor. Herein, we report an adenocarcinoma of the seminal vesicle. The patient was a 50-year-old man who complained of a two-month history of terminal hematuria and lower abdominal discomfort. A pelvic magnetic resonance imaging study. Indicated a seminal vesicle cyst with focal intraluminal growth. Right seminal vesiculectomy was performed. Grossly, the seminal vesicle revealed a markedly cystic change with focal, friable, intraluminal papillary growth. The intraluminal mass showed a noninvasive adenocarcinoma with a tubular and tubulopapillary pattern, accompanied by necrosis. Immunohistochemically, the tumor was positive for cytokeratin and carcinoembryonic antigen and negative for prostate specific antigen and prostatic acid phosphatase.

A Case of Multiple Primary Malignancy / 대한산부인과학회잡지

Multiple primary cancer means that more than two cancers are independently developed in one individual. The frequency of multiple primary neoplasm is increasing as a result of the lengthening average lifetime, progress in cancer screening technique, and improved cancer therapy. Gynecologic malignancies are especially prone to associated with primary neoplasms elsewhere. The frequency of multiple primary cancers was significantly higher in patients with uterine corpus cancer than in those suffering from cervical cancer. This case has different cell types of cancer in uterine cervix, thyroid and colon simultaneously. So, we report the case with a brief review of literatures.

Fine Needle Aspiration Cytology of Anaplastic Carcinoma of the Thyroid with Osteoclast-like Giant Cells : A Case Report / 대한세포병리학회지

Multinucleated giant cells of osteoclast-like appearance can be seen in a type of anaplastic carcinoma of thyroid and only a few case reports for fine needle aspiration cytologic findings are found in the literatures. Recently, we experienced a case of anaplastic carcinoma of thyroid with many osteoclast-like giant cells in a 72-year-old woman. The cytologic features and immunohistochemical results are described with special emphasis on differential diagnosis.

Distribution of Elastic and Collagen Fiber in Uvular Tissue of Patients with Obstructive Sleep Apnea and Snorers / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Sleep related obstructive breathing disorders are frequently observed in adults. Despite extensive work of recent years, the essential pathophysiologic mechanism that mediates this process is still unclear. This study was conducted to determine whether distribution of connective tissue in the uvula is different among apneics, snorers and normal controls. MATERIALS & METHODS: Uvula was obtained by uvulopalatopharyngoplasty in 8 apneics, 6 snorers (mean apnea index: 17.1, 2.43 respectively) and by autopsy in 5 individuals not known to have snoring. The surgical specimen of uvula was fixed in 10% formalin and subsequently embedded in paraffin. Each specimen was cut into 5 micrometer-thick section and stained with hematoxylin-eosin for general appearance, Masson-Trichrome stain for collagen fiber, and Verhoeff stain for elastic fiber. Microscopic examination was performed by two pathologists who were blinded to the polysomnographic data. RESULTS: Infiltration of inflammatory cells and edema were significantly increased in the snorer and apneic groups compared with the control group (p<0.0001, p<0.05 respectively). Compared with the normal group, the density of elastic fibers and collagen fibers were significantly decreased in snorers and apneics (elastic fiber: p<0.0005, collagen fiber: <0.01). The distribution of elastic and collagen fiber showed significantly heterogeneous patterns in the snorer and apneic group compared to the control group (elastic fiber: p<0.001, collagen fiber: p<0.0005). CONCLUSION: Since connective tissue fibers have the supportive function in the body, this condition may result in increased collapsibility of upper airway and contributes to the development of obstructive sleep apnea.

Expression of CD44v6 Protein in the Progression of Colorectal Carcinomas

During tumor progression, a subset of cells acquires metastatic properties, presumably through a series of genetic alterations. CD44 variant glycoproteins containing sequences encoded by exon v6 are related to tumor progression of human colorectal cancer. But their expression in normal colonic epithelium is controversial and studies of CD44 on each step of colorectal carcinogenesis are scanty. We studied CD44v6 expression in the normal colonic mucosa, adenoma, carcinoma in situ, and invasive colorectal carcinomas of different Astler-Coller stages. Endoscopically or surgically resected 36 normal colonic mucosa, 19 adenomas, 8 cases of carcinoma in situ, and 25 cases of carcinoma were selected. After immunohistochemical stain with CD44v6 antibody, positivity was graded as 0 to 4 based on the estimated percentage of positively stained tumor cells. The intensity of positive staining cells was also graded as 0 to 3. In all but one cases (97.2%), normal colorectal mucosa was negative for CD44v6. Positive rates in adenoma, carcinoma in situ, Astler-Coller stage A/B and C/D carcinoma were 73.6%, 88.9% and 87.5%, respectively. There was no statistically significant difference in the positivity between these groups. The staining intensity was significantly higher in the cases of stage C/D carcinoma group than those of adenomas (p<0.05). The percentage of positivity for CD44v6 was higher in stage C/D carcinoma group than adenoma, carcinoma in situ, and stage A/B carcinoma group (p<0.05). Expression of CD44v6 in the normal colonic mucosa was extremely rare and the positivity was increased according to the progression of colorectal tumors. Furthermore, it is more important to interpret the CD44v6 positivity according to the estimated percentage of positively stained tumor cells.

Trichofolliculoma in Korean Patients: A report of six cases

Trichogenic tumors are a very rare group of primary cutaneous neoplasms and a few tumors have been recognized. Trichofolliculoma is a benign, papular, organoid adnexal tumor of the skin and presents as a solitary skin-colored nodule. We report six cases of trichofolliculoma occurred in Korean patients consisting of three male and three female patients. The duration of disease was variable from 3 months to 5 years. The site of tumor was limited to the face and included eyelid in two cases, nose in two cases, cheek and posterior aspect of ear in each one case. The size of the mass varied from 4 to 8 mm in diameter. Microscopically, all the cases showed large central cystic hair follicle with numerous secondary follicular buds, producing a radiating pattern. The stroma was fibrotic and well demarcated from the adjacent dermis.

A Case of Primary Bone Marrow Lymphoma Secondary to Aplastic Anemia / 대한혈액학회지

Primary bone marrow lymphomas are infrequent. We report a case of primary bone marrow T-cell lymphoma in a 33-year old patient who has been treated as a hypocellular marrow. There was no enlarged lymph nodes or organomegaly. The peripheral blood smears showed several lymphoid atypical cells. The bone marrow biopsy revealed markedly increased cellularity and disclosed malignant lymphoma, diffuse, large-cell, immunoblastic type The lymphoma cells disclosed positive reaction in leukocyte common antigen (CD45) and UCHL-1 (CD45RO) antibodies which is compatible with a T-cell phenotype. The patient was received allogeneic bone marrow transplantation.

Fine Needle Aspiration Cytology of Solid Type Adenoid Cystic Carcinoma of Buccal Mucosa: A Case Report / 대한세포병리학회지

Adenoid cystic carcinoma constitutes 4 percent of all benign and malignant epithelial salivary gland tumors and is a highly malignant tumor of the salivary glands. The cytologic presentation in aspirates is usually characteristic with spherical clusters(balls) of small tumor cells filled with hyaline material. But in case of the poorly differentiated variety(solid type), it is difficult to differentiate from other tumors because sheets of small, fairly monotonous malignant cells, with somewhat larger and more conspicuous nuclei are only seen. The cytologic findings of fine needle aspiration of solid type adenoid cystic carcinoma of buccal mucosa in a 51-year-old man are presented. On cytologic findings, solid sheets of monotonous tumor cells with focal necrosis was noted on a hemorrhagic background and the characteristic cytologic features of adenoid cystic carcinoma was absent.

A Case of Primary Pulmonary Sarcoma with Morphologic Features of Biphasic Synovial Sarcoma / 결핵

Synovial sarcoma is a malignant soft tissue neoplasm that occurs frequently in the extremities of young adults, near large joints. The lung is a common site of metastasis but an extremely unusual primary site for synovial sarcoma. We report an unusual case of primary synovial sarcoma that arose in the lung of a 59-year-old woman. The tumor had histologic and immunophenotypic features consistent with biphasic synovial sarcoma. These features included of an intimate admixture of cytokeratin and epithelial membrane antigen(EMA)-positive neoplastic epithelial cells and vimentin-positive fibroblast-like spindle cells. The patient had a closed thoracomy drainage and doxycycline pleurodesis for malignant loculated effusion and showed tumor extension in the left whole lung 4 months after pleurodesis. This case is an usual addition to the small number of published reports on primary pulmonary synovial sarcoma. The distinctive features of this neoplasm allow it to be distinguished from a variety of primary and metastatic malignancies in the lung.