• Korean Journal of Radiology
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• 제22권6호
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• pp.944-950
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• 2021

Objective: To demonstrate the feasibility of percutaneous microwave ablation in desmoid fibromatosis with respect to tumor volume control and improvement in the quality of life. Materials and Methods: Twelve microwave ablations were performed in 9 patients with a histological diagnosis of desmoid fibromatosis between January 2010 and January 2019. The study population included 6 female and 3 male, with an age range of 21-76 years (mean = 46.6 years; standard deviation [SD] = 19.3 years). The mean major axis of the tumors was 10.9 cm (SD = 5.2 cm) and mean lesion volume was 212.7 cm³ (SD = 213 cm³). Their anatomical distribution was as follows: 3 lesions in the thigh, 2 in the gluteus, 2 in the leg and 2 in the periscapular region. We evaluated the reduction in tumor volume and improvement in the quality of life based on the Eastern Cooperative Oncology Group (ECOG) scale. Results: An average tumor volume reduction of 70.4% (SD = 24.9) was achieved, while the quality of life (ECOG scale) improved in 88.9% of patients. Conclusion: Percutaneous microwave ablation may potentially be a safe, effective, and promising technique for controlling tumor volume and improving the quality of life in patients with desmoid fibromatosis.

• Journal of Chest Surgery
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• 제49권2호
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• pp.134-137
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• 2016

A mass excision surrounding the bronchial stump was performed to exclude malignancy in a 42-year-old man who had undergone a right lower lobectomy for lung cancer. The mass was identified as a cicatricial fibroma. Cicatricial fibromatosis, which is desmoid fibromatosis that arises in a surgical scar, is a well-known clinical condition. It consists of histologically benign neoplasms. Their occurrence after thoracic surgery is extremely rare. Biopsy or excision of suspicious lesions is very important for diagnosis. R0 resection remains the principal outcome for intrathoracic desmoid fibromatosis. We report that a cicatricial fibromatosis in the subcarinal space was removed after suspicion of local recurrence at the bronchial stump follwing lobectomy for lung cancer.

• 대한영상의학회지
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• 제82권5호
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• pp.1297-1303
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• 2021

췌장의 충돌 종양은 매우 드문 종양으로서 췌장선암종과 신경내분비 종양, 췌관내유두상 점액 종양과 신경내분비 종양, 그리고 췌장 고형성 가유두상 종양으로 이루어진 증례들이 보고된 바 있다. 우리는 30세 임신한 여성에서 빠르게 자란, 데스모이드 섬유종증과 점액성 낭성종양으로 이루어진 췌장의 충돌 종양의 증례를 보고하고자 한다. 저자들이 아는 한, 섬유종증과 점액성 낭성 종양으로 이루어진 췌장의 충돌 종양을 최초로 보고하는 증례이다.

• Tuberculosis and Respiratory Diseases
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• 제67권5호
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• pp.449-453
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• 2009

Desmoid tumor (fibromatosis) is a histologically benign fibrous neoplasm showing locally infiltrating growth. This type of tumor commonly occurs in the abdomen, but intrathoracic desmoid tumor is uncommon. To date, 12 cases of intrathoracic desmoid tumor protruding into the pleural cavity, radiologically mimicking pleural masses, have been reported. Here, we report on a case of intrathoracic desmoid tumor protruding into the pleural cavity, and partially covered by parietal pleura. The main preoperative differential diagnoses included pleural solitary fibrous tumor, inflammatory pseudotumor or malignant mesothelioma. A near-total mass excision was performed. Pathologically, the tumor was composed of a paucicellular arrangement of spindle-shaped cells with fibromyxoid stroma. The resection margin was partially involved with spindle cells present. On histochemical staining, the spindle cells were strongly positive for vimentin and negative for CD34, consistent with a desmoid tumor. The patient was stable without further adjuvant treatment during 6-years of follow-up.

• 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.

• 대한골관절종양학회지
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• 제1권2호
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• pp.205-209
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• 1995

A desmoid tumor is a locally aggressive growth of connective tissue origin which infiltrates the surrounding tissue and has a marked tendency for recurrence. And so it was also called as an aggressive fibromatosis, musculofascial fibromatosis or fibrosarcoma etc. Thirteen cases of desmoid tumor was treated since 1980, and their retrospective study was done with 79 months of follow-up after initial surgical excision. The female was involved in 12 cases(one male) with the age ranged from 7-50 years, average 28 years, and seven patients in third decade. A slowly growing mass was excised on average 4 months after first notice of the mass, but their margins are not demarcated clearly in most cases. Wide excision in 12 cases was done, but wide excision and saphenous vein graft was performed in one case because of invasion of posterior tibial artery by tumor mass. The tumor was found on extraabdominal region in 8 cases(61.5%) but 5 cases in abdominal wall(38.5%). The recurrence rate was high(6/13, 46.2%), and 11 times in 6 patients were recurred(average 1.8 times), within 27 months of initial excision. Six cases of recurrence were treated with wide excision again in 3 cases, wide excision combined with radiotherapy(4,000-6,000cGy) in 4 cases and wide excision with chemotherapy in one case. During the follow-up for average 21 months after treatment, no recurrences are found. Tumor remission periods without recurrence are average 67 months in all, and 11 years in longest case. Histologically it was very mimic with fibrosarcoma but could be differentiated with Trichrome stain, and their findings are not changed after recurrence.

• 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.

• 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.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제16권2호
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• pp.186-195
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• 1994

Fibromatosis is benign fibroblastic proliferative lesion with abundant collagenous neo-formation located principally in the abdominal wall and in the upper and lower extremities (Masson & Soule, 1966). Wilkins and Waldron, in 1975, suggested that the title aggressive fibromatosis was a more appropriate term, reflecting the invasive characteristics of the disease. Synonyms listed were extra-abdominal desmoid, juvenile fibromatosis, aggressive infantile fibromatosis and congenital fibrosarcoma. A total of 12% of all fibromatosis arise in head and neck. Fibromatosis of the oral cavity is uncommon and is even more rare when in involve the mandibule. It is a locally aggressive fibrous tissue tumor, generally does not metastasize, but may cause considerable morbility and even death due to local infiltration. The degree of microscopic cellularity is variable, not only from tumor to tumor but also from area to area in the same tumor. Some tumors present with proliferation of mature fibroblasts and a dominating collagenous component : others may show a lack of the tumor in both types. The common histologic denominator appears to be cellular interlacing bundles of elongated fibroblasts, showing little or no mitotic activity and no pleomorphism. Mitosis are not a consistent index of malignancy when found in younger age groups. Fibromatosis still posses difficult problems of diagnosis and treatment. It is frequently recurrent and infliltrates neighbouring tissues. These lesion infliltrate widely and replace muscle, fat, and even bone with fibrous tissue of varying cellularity. Lesion representing fibromatosis in the oral cavity must be carefully evaulated by both surgeon and pathologists to ensure proper diagnosis and treatment planning. When these lesions involve bone, surgeon must be aware of the lesion's potential to perforate the cortex and expand while remaining hidden from the surgeon's view. Careful and precise clinical correlation with histologic appearance is essential to preclude misdiagnosis of fibrosarcoma yet provide surgical treatment plan that provides adequate local excision and long-term follow up. As regards cause, little is known. It is attributed to trauma or alteration in the sex hormone(Carlos, et al, 1986). Clinially, the lesion is reported to be not painful in most cases, but capable of rapid growth. The treatment is essentially surgical excision with wide margin of adjacent uninvolved tissue. Radiotherapy, hormone treatment or chemotherapy are of no use (WIkins et al, 1975 ; Majumudar and Winiarkl, 1978). We report a case of aggressive fibromatosis of 15-year-old with a lesion in the soft tissue of the parotid area that invaded the underlying bone of the mandibular body.

• 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.