• Journal of Yeungnam Medical Science
• /
• v.33 no.1
• /
• pp.44-47
• /
• 2016

Primary dissection of the renal artery is rare. Spontaneous renal artery dissection can be associated with diseases such as medial degeneration, neurofibromatosis, syphilitic arteritis, tuberculosis, polyarteritis nodosa, Marfan syndrome, fibromuscular dysplasia, or Ehlers-Danlos syndrome (EDS). Among these causes, EDS related renal artery dissection is very rare worldwide and has not been previously reported in Korea. EDS are a group of heritable connective tissue disorders characterized by fragility of the skin and hypermobility of the joints. We describe the case history of a young man who presented with left side flank pain, hypermobility of the hand joints and showed left renal artery dissection on computed tomography and angiography that turned out to be the first complication of vascular type EDS.

• Archives of Reconstructive Microsurgery
• /
• v.22 no.2
• /
• pp.78-81
• /
• 2013

Neurofibroma may present as a solitary lesion or as multiple lesions. Although there is no site of predilection for solitary lesions, occurrence on the hand is rare. Plexiform neurofibroma can develop in isolation or more commonly as a part of neurofibromatosis type 1. In those that apper in isolation, trauma has been suggested as a precipitating factor. A 68-year-old male farmer had experienced repetitive prior episodes of trauma in the involved finger. He presented with a painless mass on the dorsal aspect of the fifth finger. Physical examination showed a protruding mass measuring approximately $15{\times}20mm$ which was not tenderness to palpation and any skin changes or pigmentation. Ultrasonography showed a cystic mass on the dorsal aspect of the middle phalanx. Microsurgical dissection was applied in order to seperated the lesion from the ulnar side of the dorsal branch of the digital nerve. Pathologic examination of the specimens revealed neurofibroma. At three-month follow-up, motor and sensory function were intact, and range of motion was fully recovered. Traumatic solitary neurofibroma is a rare tumor of the hand, especially in the finger. Hand surgeons should be aware of the diagnostic possibilities of this tumor based on examination, history taking and imaging studies.

• Archives of Plastic Surgery
• /
• v.38 no.6
• /
• pp.886-889
• /
• 2011

Purpose: Malignant peripheral nerve sheath tumor without neurofibromatosis type 1 is very rare neoplasm. Development in the superficial soft tissue is exremely rare. Authors experienced one rare case of primary malignant peripheral nerve sheath tumor developed on abdomen. The clinical and histologic findings were described. Methods: An 83-year-old man visited hospital with an $11{\times}6.5{\times}4.5$ cm sized ulcerated and hemorrhagic mass on abdomen. The tumor was localized in abdominal skin and started growing 3 years ago. Results: Wide excision with safety margin of 2 cm and limberg flap was done. The postoperative biopsy revealed a malignant peripheral nerve sheath tumor. There was no evidence of recurrence of tumor for 16 months. Conclusion: Malignant peripheral nerve sheath tumor is an aggressive malignant tumor. An abrupt enlargement of size, ulceration and bleeding are suggestive of malignant chnages of the tumor. We recommand early wide excision with enough safety margin as treatment of malignant peripheral nerve sheath tumor.

• Journal of Life Science
• /
• v.28 no.9
• /
• pp.1100-1117
• /
• 2018

The Ras superfamily of small G-proteins acts as a molecular switch on the intracellular signaling pathway. Upon ligand stimulation, inactive GTPases (Ras-GDP) are activated (Ras-GTP) using guanine nucleotide exchange factor (GEF) and transmit signals to their downstream effectors. Following signal transmission, active Ras-GTP become inactive Ras-GDP and cease signaling. However, the intrinsic GTPase activity of Ras proteins is weak, requiring Ras GTPase-activating protein (RasGAP) to efficiently convert RAS-GTP to Ras-GDP. Since deregulation of the Ras pathway is found in nearly 30% of all human cancers, it might be useful to clarify the structural and physiological roles of Ras GTPases. Recently, RasGAP has emerged as a new class of tumor-suppressor protein and a potential therapeutic target for cancer. Therefore, it is important to clarify the physiological roles of the individual GAPs in human diseases. The first RasGAP discovered was RASA1, also known as p120 RasGAP. RASA1 is widely expressed, independent of cell type and tissue distribution. Subsequently, neurofibromatosis type 1 (NF1) was discovered. The remaining GAPs are affiliated with the GAP1 and synaptic GAP (SynGAP) families. There are more than 170 Ras GTPases and 14 Ras GAP members in the human genome. This review focused on the current understanding of Ras GTPase and RasGAP in human diseases, including cancers.

• Clinical and Experimental Pediatrics
• /
• v.45 no.11
• /
• pp.1417-1421
• /
• 2002

Scoliosis describes a lateral curvature of the spine and is often associated with cosmetic and functional impairments due to severe deformity of the spine. The incidence of adolescent scoliosis is 2-4% of children between 10 and 16 years of age. Eighty five percent of them are idiopathic, in which the most common type of scoliosis is right side-bending. In addition, it is classified into congenital, and secondary scoliosis such as neuromuscular disease and neurofibromatosis. Congenital scoliosis is associated with abnormalities of urinary system(20%), congenital heart diseases(15%), and other abnormalities(10%) such as syringomyelia. We experienced a case of scoliosis by a foreign body(a pencil) in the colon which has never been reported up to date. A brief review of the literature was made.

• Korean Journal of Otorhinolaryngology-Head and Neck Surgery
• /
• v.55 no.3
• /
• pp.181-184
• /
• 2012

The malignant peripheral nerve sheath tumor is an extremely rare soft tissue sarcoma. It is a highly malignant sarcoma, which is locally invasive, frequently leading to multiple recurrences and eventual metastatic spread. The peak incidence of disease is known to occur sporadically between the age of 20s and 50s, and is usually associated with the neurofibromatosis type I. In human body, the trunk and extremities are the most commonly involved sites, with only 8-14% of all lesions appearing in the head and neck region. We present a case of malignant peripheral nerve sheath tumor involving the right parapharynx in a 48-year-old patient who complained of headaches in the right parietal area and of dysphagia that aggravated over a month. After surgery, tumor was finally diagnosed as malignant peripheral nerve sheath tumor by histopathologic examinations. The authors report a case of malignant peripheral nerve sheath tumor in the right parapharynx with a review of the literature.

• The Korean Journal of Food And Nutrition
• /
• v.10 no.1
• /
• pp.74-81
• /
• 1997

In this study, we tried to figure out how phenol effects on cancer prevention, and for this purpose we focused on phenol effects on TBARS and the relationship between TBARS(thiobarbituric acid-reactive substances) and cancer. A protocol using a nutritionally adequate amino acid-based diet and a transgenic mouse model of neurofibromatosis was used to evaluate the effect of dietary phenols on body tissue oxidation and tumor onset. The mice carry the human T-lymphotropic virus type-1 transactivator(texl) gene and spontaneously develop externally visible tumors. Twenty-five male transgenic mice were systematically assigned into five groups, control group, 2 mmol, 4 mmol, 8 mmol catechin/kg diet groups and wine solid group. Mice in control group were without catechin, Mice in wine solid group received red wine 750 mL/kg diet, Mice were examined daily, and the age at which a first tumor appeared was recorded. Transgenic mice consuming catechin and wine solid were older when a first tumor appeared. No tumor was found in one mouse of 4 mmol catechin/kg diet and one mouse of 8 mmol catechin diet group. Levels of TBARS in brain and spleen of 8 mmol catechin group and wine solid group were significantly decreased as compared to the same tissue in control group. TBARS levels in tissues were significantly correlated with tumor onset. Results from this study suggest that dietary phenol effects on cancer prevention through tissue antioxidation in spite of different kinds of phenols.