• Journal of Genetic Medicine
• /
• v.6 no.1
• /
• pp.91-94
• /
• 2009

Werner syndrome (WRN), or adult progeria, is a very rare, autosomal recessive disorder characterized by the appearance of accelerated aging, including cataracts, gray hair, skin atrophy, and atherosclerosis. This syndrome is caused by mutations in the WRN gene and had a high risk of a spectrum of rare neoplasms including: i) non-epithelial malignant or pre-malignant tumors/conditions, osteosarcomas and soft tissue sarcomas, malignant melanomas, myeloid leukemia and myelodysplastic syndrome; ii) an epithelial neoplasm, thyroid carcinoma, and iii) meningiomas. Recently, authors experienced a case of Werner syndrome complicated by bone metastasis of rhabdomyosarcoma in a 20-year old Korean man. The patient revealed a painful mass on his right knee and progeroid features, short stature, scalp alopecia, abnormal dentition, craniofacial disproportion, hypothyroidsm, cataracts and osteoporosis. The onset of symptoms of Werner syndrome generally precedes any later symptoms of associated conditions, such as malignant tumor. Therefore, early recognition of Werner syndrome is important to assist identification of malignant tumors at an early stage in this patient group.

• The Korean Journal of Zoology
• /
• v.17 no.2
• /
• pp.51-56
• /
• 1974

Newborn Armenian and Chinese hamsters inoculated with adenovirus type 12 developed undifferentiated small cell tumors as early as 27 days after inoculation in the Chinese hamsters and within 30$\\sim$45 days in the Armenian hamsters. These tumors were transplantable and epithelial-like cell in morphology. Cultures of 6 tumors underwent spontaneous reversion to fibroblast-like morphology during the 14$\\sim$20 in vitro passages in the absence of chromosomal disturbances. While epithelial-like tumor derivatives were oncogenic and positive for the T-antigen, fibroblast-like revertants were non-oncogenic and negative for the T-antigen. Two other tumor derivatives reverted to fibroblast-like forms, immediately following exposure to SV40. These lacked the adenovirus T-antigen but were positive for the SV40 T-antigen and formed sarcomas in animals.

• The Journal of the Korean bone and joint tumor society
• /
• v.5 no.4
• /
• pp.208-215
• /
• 1999

The purpose of this study was to identify the independent clinicopathologic prognostic factors of soft tissue sarcoma affecting local recurrence, metastasis and survival. Retrospectively collected data from 130 patients with soft tissue sarcoma were analyzed. Patient, tumor and pathologic factors were analyzed by univariate and multivariate methods for the endpoints of local recurrence, metastasis and survival. In univariate analysis, wide surgical margin, adjuvant radiotherapy and age younger than 40 years reduced local recurrence. Tumor size larger than 5cm was related with a higher rate of metastasis. Patients with metastasis at initial presentation and with a large tumor size had a low survival rate. In multivariate analysis, adjuvant radiotherapy and young age were significantly correlated with a low local recurrence rate. In conclusion, patients with metastasis at initial presentation and a large tumor size had a reduced survival rate. Independent adverse prognostic factors for local recurrence were old age and not undergoing adjuvant therapy.

• Asian Pacific Journal of Cancer Prevention
• /
• v.17 no.1
• /
• pp.81-83
• /
• 2016

Background: Cancer is a subject of continuing concern, more common in adults than in children, but often with a poor outcome in the latter. Our study set itself the objective to describe the epidemiological and histological aspects of solid cancers in children in Togo. Materials and Methods: This descriptive, cross-sectional study focused on cases of solid cancers in children diagnosed from 2010 to 2014 (5 years) at the pathology laboratory of the Tokoin teaching hospital. Data were collected from the records of that laboratory. Results: We collected 66 cases of childhood cancer representing 5% of all solid cancers. The annual incidence was 13.2 cases. The sex ratio (M/F) was 1.4; mean age was of $7.2{\pm}1.6years$. The age group most affected was that of 5-9 years (40.9%). Four histological groups of solid childhood cancers were listed: lymphoma (n=34 cases; 51.5%), embryonic cancer (n=17 cases; 25.8%), sarcomas (n=13 cases; 19.7%) and carcinoma (n=2 cases; 3%). The most common histological types were Burkitt lymphoma (36.4%), nephroblastoma (10.6%) and retinoblastoma (10.6%). Conclusions: This study shows that solid cancers in children are relatively frequent in Togo with a male predominance. They are still largely dominated by Burkitt lymphoma, followed by retinoblastoma and nephroblastoma.

• Journal of Yeungnam Medical Science
• /
• v.28 no.1
• /
• pp.70-76
• /
• 2011

Rhabdomyosarcomas are soft tissue sarcomas; while extremely rare in adults, they are one of the most common neoplasms in children and adolescents. Histologically, they can be classified into embryonal (ERMS), alveolar (ARMS), pleomorphic, and undifferentiated types. The ARMS type is very rare, and is associated with a poor prognosis. Common primary sites of ARMS are the trunk and extremities. We report on a case of paraaortic, supraclavicular, and axillary lymph node metastasis from paratesticular ARMS treated with VAC (vincristine, dactinomycin, cyclophosphamide)/ IE(ifosfamide, etoposide) chemotherapy in a young adult. Administration of six cycles of chemotherapy with VAC/IE resulted in complete remission. The patient has maintained complete remission over the past 27 months.

• Asian Pacific Journal of Cancer Prevention
• /
• v.16 no.8
• /
• pp.3419-3423
• /
• 2015

Background: Liposarcoma of the spermatic cord is rare and frequently misdiagnosed. The standard therapeutic approach has been radical inguinal orchiectomy with wide local resection of surrounding soft tissues. The current trend of organ preservation in the treatment of several cancers has started to evolve. Herein we present our testis-sparing surgery experience in the treatment of spermatic cord liposarcoma and a pooled analysis on this topic. Materials and Methods: Clinical information from patient receiving organ-sparing surgery was described. Clinical studies evaluating this issue were identified by using a predefined search strategy, e.g., Pubmed database with no restriction on date of published papers. The literature search used the following terms: epidemiology, surgery, chemotherapy, radiotherapy, testis sparing surgery, spermatic cord sarcomas/liposarcomas. Results: Patient received a complete excision of the lesion, preserving the spermatic cord and the testis. The final pathological report showed a well differentiated liposarcoma with negative surgical margins and no signs of local invasion. After 2-year of follow-up, there was no evidence of local recurrence. Since the first case reported in 1952, a total of about 200 well-documented spermatic cord liposarcoma cases have been published in English literature. Among these patients, only three instances were reported to have received an organ-sparing surgery in the treatment of spermatic cord liposarcoma. Conclusions: Radical inguinal orchiectomy and resection of the tumor with a negative microscopic margin is the recommended treatment for liposarcoma of the spermatic cord. But for small, especially well-differentiated, lesions, testis-sparing surgery might be a good option if an adequate negative surgical margin is assured.

• Archives of Plastic Surgery
• /
• v.40 no.3
• /
• pp.238-243
• /
• 2013

Background Primary malignant tumors of the hand, although unusual, may present varied and often complex clinical problems. The main treatment modality of skin cancer of the hand has changed. Methods We retrospectively reviewed the medical records of 43 patients who underwent surgery for malignant skin tumors of the hand during an 18-year period, from September 1994 to February 2012. The characteristics of the tumor, methods of reconstruction, and long-term results were reviewed. Results We had 43 patients with 27 melanomas, 14 squamous cell carcinomas, and 2 sarcomas. Their ages ranged from 19 to 74 years (mean, $53.4{\pm}14.5$ years), from 46 to 79 years (mean, $59.7{\pm}9.6$ years), and from 15 to 43 years (mean, $29{\pm}19.8$ years), respectively. Thirty-four cases occurred on the fingertip (16 of those cases on the thumb), 5 cases occurred on the palm, and 4 cases on the dorsum of the hand. Amputation was most frequently used in early cases, but recently, tissue-sparing excision has been performed frequently. The incidence of local recurrence was 3 cases and distant metastasis was 1 case, and the 5-year survival rate was 100%, except in 4 cases due to follow-up loss. Conclusions The principles of treatment-to be curative and to preserve function and appearance-are important points. "Preservative surgery" preserves function and cosmesis of the involved finger or hand dorsum or palm. Preservative surgery not only emphasizes less resection and surgery of a smaller scale, but also optimal reconstruction of the soft tissue defect of the digit.

• Archives of Plastic Surgery
• /
• v.32 no.6
• /
• pp.760-762
• /
• 2005

Synovial Sarcoma is the fourth most common sarcoma, accounting for 8-10 % of all sarcomas. Synovial sarcoma is highly malignant tumor of mesenchymal origin but rarely occurres in head and neck area. Less than 100 cases of synovial sarcoma occurring in head and neck area have been reported all over the world. Pathologically, there is two type of synovial sarcoma: monophasic variant is composed of only one cell type and "classic" (biphasic) synovial sarcoma has two cellular component, a spindle cell(fibrosarcoma-like) component and a pseudoepithelioma component. Recommended treatment is wide resection with negative margins. The role of chemotherapy and radiation therapy is controversial. We experienced a 42-year-old male patient with slowly enlarging, deep seated mass on right cheek. In the first operation, we suggested that the mass maybe benign tumor. But, initial excisional biopsy specimen of the primary lesion was consistent with synovial sarcoma. The final diagnosis was monophasic synovial sarcoma which was composed of spindle cells. Radical resection was performed two months later because remnant tumor was found on follow up MRI. No further treatment was done. There were no recurrence or metastasis on follow up MRI, chest CT and whole body bone scan after 15 months. This is a report of a rare case of synovial sarcoma of the face with a literature review.

• Korean Journal of Head & Neck Oncology
• /
• v.19 no.2
• /
• pp.184-187
• /
• 2003

Synovial sarcoma is usually found near large joints and bursae of the extremities. Only 10% of all synovial sarcomas occur in the head and neck. The most common site affected in the head and neck is the hypopharynx, and very rare in the infratemporal fossa. Treatment of synovial sarcoma of the head and neck is difficult and must have multimodality which consists of operation, radiation and chemotherapy. Prognosis is associated with completeness of resection. We reported a case of synovial sarcoma arising from the infratemporal fossa, the first case in Korea, with review of literature.

• The Korean Journal of Cytopathology
• /
• v.5 no.1
• /
• pp.74-78
• /
• 1994

A case of retroperitoneal paraganglioma is presented with fine needle aspiration cytologic features. A 57-year-old woman complained of abdominal discomfort and left flank pain for 2 years. The abdominal CT scan revealed an ovoid mass left to the abdominal aorta. Percutaneous fine needle aspiration was performed from the mass. The smear revealed cellular sheets or groups on hemorrhagic background. The tumor cells were ovoid, round to spindle shaped with mild to moderate cellular pleomorphism. The nuclei were round to ovoid and had evenly dispersed chromatin and small nucleoli. The cytoplasm was amphophilic, finely granular and poorly defined. Cells having large or spindle nuclei were quite frequently observed, however, mitosis was not present The cytologic findings suggested paraganglioma, but the frequent spindle cell pattern and the pleomorphism made it difficult to exclude other endocrine tumors and sarcomas. The clinical, histological and ultrastructural findings as well as cytologic findings contributed to confirmatory diagnosis.