• Imaging Science in Dentistry
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• 제45권3호
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• pp.181-185
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• 2015

Numerous cases of enamel renal syndrome have been previously reported. Various terms, such as enamel renal syndrome, amelogenesis imperfecta and gingival fibromatosis syndrome, and enamel-renal-gingival syndrome, have been used for patients presenting with the dental phenotype characteristic of this condition, nephrocalcinosis or nephrolithiasis, and gingival findings. This report describes a case of amelogenesis imperfecta of the enamel agenesis variety with nephrolithiasis in a 21-year-old male patient who complained of small teeth. The imaging modalities employed were conventional radiography, cone-beam computed tomography, and renal sonography. Such cases are first encountered by dentists, as other organ or metabolic diseases are generally hidden. Hence, cases of amelogenesis imperfecta should be subjected to advanced diagnostic modalities, incorporating both dental and medical criteria, in order to facilitate comprehensive long-term management.

• Archives of Plastic Surgery
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• 제33권1호
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• pp.124-126
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• 2006

Pachydermodactyly is a rare distinct form of fibromatosis characterized by acquired, asymptomatic dense fibrosis and fibroblastic proliferation around one or more proximal interphalangeal joints. Intralesional triamcinolone injection may be helpful in improving the clinical appearance. A 20-year-old unemployed female patient gave a four years history of progressive swelling of the fingers affecting predominantly the proximal interphalangeal joints of index fingers of left hand and index, middle fingers of right hand. There was no pain and tenderness. Physical examination, complete blood count, antinuclear antibody, rheumatoid factor were unremarkable and radiograph of both hands showed soft tissue swelling only without bony abnormality. We excised the redundant soft tissue from the both sides of proximal interphalangeal joint with longitudinal elliptical fashion. After operation, the fingers of the patient showed marked improvement cosmetically. There were no motor or sensory changes. Pachydermodactyly was first reported in 1996 in Korea. Histologic features include an increased dermal accumulation of collagen and occasionally an increased number of fibroblast. We report for one patient with pachydermodactyly treated with longitudinal elliptical excision and obtained an improvement of clinical appearance.

• 치과방사선
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• 제29권1호
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• pp.357-365
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• 1999

Desmoplastic fibroma is a rare. benign intraosseous fibroblastic tumor. which is locally aggressive. It is osseous counterpart of soft tissue fibromatosis. The authors experienced the patient who complained persistent mouth opening limitation with mild swelling on the left mandibular angle area. After careful analysis of clinical. radiological and histopathological findings. we diagnosed as desmoplastic fibroma of the mandible. The results were as follows: 1. Main clinical symptoms were mouth opening limitation which had been persistent for 9 months and mild swelling on the left mandibular angle area. 2. Radiographs showed the radiolucent lesion and expansion of lingual cortex. CT finding is homogeneous soft tissue mass with expansion of left mandibular ramus. Destruction of medial wall of ramus and invasion to adjacent soft tissue is also seen. 3. Histopathologically, plump spindle shaped fibroblasts arranged in bundles or fascicles are observed. The cells of tumor are infiltrating into muscle fiber with destruction of bony trabeculae and merged with surrounding salivary gland.

• Journal of Yeungnam Medical Science
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• 제12권2호
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• pp.386-392
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• 1995

데스모이드 종양은 섬유아세포양 증식을 특징으로 하는 심부 섬유종증으로 조직학적으로 양성이지만 침습적인 성장 양상과 높은 재발율을 보인다. 방사선학적으로 초음파상 경계가 비교적 뚜렷한 고형종괴로 보이며 내부의 에코는 비특이적이며 CT 소견상 근섬유의 주행방향을 따라 길쭉한 모양을 보이고 조영제 주입전에는 주변 근육과 비슷한 등음영 또는 약간 저음영을 보이고 조영후에는 시간이 감에 따라 점차 조영증강되는 소견을 보인다. 저자들은 급속조영 CT를 시행하여 시간에 따른 조영 양상을 관찰함으로써 종괴의 조직학적 구성을 짐작할 수 있었고 병리조직학적으로 확진된 데스모이드 종양 2례를 경험하였기에 보고하는 바이다.

• 대한족부족관절학회지
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• 제14권2호
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• pp.169-172
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• 2010

Purpose: The authors have experienced various lesions that simulate ganglion of the foot on the ultrasonography. The purpose of this study is to evaluate ultrasonographic findings of soft tissue lesions, which were interpreted as ganglion but confirmed as different lesions in the foot. Materials and Methods: We reviewed a database of patients with ganglion on the ultrasonography from two different institutions. There were 109 patients who underwent both ultrasonography and surgical confirmation. Twenty one lesions were identified, of which initial interpretation on the ultrasonography included ganglion which pathology revealed to be different lesions. All images were evaluated by one musculoskeletal radiologist, regarding size, margins, internal echogenicity of lesions, and presence of posterior enhancement. Results: Of 21 lesions, there were 6 fibrous tumors including fibroma, giant cell tumor of tendon sheath, and fibromatosis, 3 hemangiomas, 2 epidermal inclusion cysts, 2 chondromas, 2 angioleiomyomas, 1 trichilemal cyst, 1 neurofibroma, 1 granular cell tumor, 1 neurilemmoma, 1 neuromyxoma, and 1 nodular hidradenoma. Mean size of the lesion was 1.1 cm. Margins were smooth in 10, mild lobulation in 8 and marked lobulation in 3 lesions. Lesions were hypoechoic in 16, anechoic in 4 and isoechoic in 1 case. Posterior acoustic enhancement was definitely present in 5 lesions. Conclusion: On the ultrasonography, various soft tissue lesions of the foot may be confused with ganglion. During surgical resection care should be given even to a simple ganglion as it might turn up to be a solid lesions such as fibrous tumors.

• Tuberculosis and Respiratory Diseases
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• 제78권3호
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• pp.267-271
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• 2015

Desmoid tumors are rare soft tissue tumors considered to have locally infiltrative features without distant metastasis until now. Although they are most commonly intraabdominal, very few cases have extra-abdominal locations. The origin of intrathoracic desmoid tumors is predominantly the chest wall with occasional involvement of pleura. True intrathoracic primary desmoid tumors with no involvement of the chest wall or pleura are extremely rare. We recently experienced a case of true intrathoracic desmoid tumor presenting as multiple lung nodules at 13 years after resection of a previous intraabdominal desmoid tumor.

• 대한소아치과학회지
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• 제28권1호
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• pp.171-174
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• 2001

결합조직형성 섬유종(desmoplastic fibroma)은 국소적으로 공격성을 보이는 골내 양정종양으로, 연조직에 발생하는 섬유종증(fibromatosis)이나 유건종(desmoid tumor)의 골내 대조병변이다. 이 종양은 대개 30세 이하의 비교적 어린 나이에 발생하며, 가장 흔한 주소와 증상은 구강 내와 구강 외의 무통성 종창으로 안모변형을 일으킨다. 환아는 약 한달 전부터 시작된 좌측 안면부 종창을 주소로 본원에 내원한 9세 남아로 종창부위를 만져보면 통증은 있지만, 악하 림프절 증대는 없었다. 파노라마 사진에서 #33과 #34 치아 사이에 비교적 경계가 분명한 방사선 투과상 병소가 관찰되었다. 임상적으로 좌측 하악체 부위에는 양성 치성종양이, #46 치관 부위는 함치성 낭이 있는 것으로 추정하고 구강악안면외과에 의뢰하여 종양 및 낭 제거술을 시행하였다. 병리조직학적으로, 종양은 방추형의 섬유모세포의 증식과 교원섬유의 침착이 특징이었다. 이 증례에서 치아 및 악골이 발육중인 어린이의 경우 안모 손상을 초래할수 있어 광범위한 외과술식은 피하였다. 그러나 이 종양의 재발율이 높은 점을 고려하여 세심하고도 철저한 추적관찰이 필요하며, 추후 재발 시에는 악골 절제도 고려해야 할 것으로 생각한다.

• Investigative Magnetic Resonance Imaging
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• 제21권3호
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• pp.162-170
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• 2017

Purpose: To evaluate the imaging findings of desmoid tumors using various imaging modalities and to evaluate whether diffusion-weighted imaging (DWI) can help differentiate between desmoid and malignant tumors. Materials and Methods: The study included 27 patients with pathologically confirmed desmoid tumors. Two radiologists reviewed 23 computed tomography (CT), 12 magnetic resonance imaging (MRI) and 8 positron emission tomography-computed tomography (PET-CT) scans of desmoid tumors and recorded data regarding the shape, multiplicity, size, location, degree of enhancement, and presence or absence of calcification or hemorrhage. The signal intensity of masses on T1- and T2-weighted imaging and the presence or absence of whirling or band-like low signal intensity on T2-weighted imaging were recorded. The apparent diffusion coefficient (ADC) values of the desmoid tumors in nine patients with DWIs were compared with the ADC values of 32 malignant tumors. The maximum standardized uptake value ($SUV_{max}$) on PET-CT images was measured in 8 patients who underwent a PET-CT. Results: The mean size of the 27 tumors was 6.77 cm (range, 2.5-26 cm) and four tumors exhibited multiplicity. The desmoid tumors were classified by shape as either mass forming (n = 18), infiltrative (n = 4), or combined (n = 5). The location of the tumors was either intra-abdominal (n = 15), within the abdominal wall (n = 8) or extra-abdominal (n = 4). Among the 27 tumors, 21 showed moderate to marked enhancement and 22 showed homogeneous enhancement. Two tumors showed calcifications and one displayed hemorrhage. Eleven of the 12 MR T2-weighted images showed whirling or band-like low signal intensity areas in the mass. The mean ADC value of the desmoid tumors ($1493{\times}10^{-6}mm^2/s$) was significantly higher than the mean of the malignant soft tissue tumors ($873{\times}10^{-6}mm^2/s$, P < 0.001). On the PET-CT images, all tumors exhibited an intermediate $SUV_{max}$ (mean, 3.7; range, 2.3-4.5). Conclusion: Desmoids tumors showed homogenous, moderate to marked enhancement on CT and MRI scans and a characteristic whirling or band-like pattern on T2-weighted images. DWI can be useful for the differentiation of desmoid tumors from malignant soft tissue tumors.