• Title/Summary/Keyword: Desmoid fibromatosis

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Percutaneous Microwave Ablation of Desmoid Fibromatosis

  • Alberto Martinez-Martinez;Jade Garcia-Espinosa;Antonio Jesus Lainez Ramos-Bossini;Fernando Ruiz Santiago
    • Korean Journal of Radiology
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    • v.22 no.6
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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.

Cicatricial Fibromatosis Diagnosis after Suspected Local Recurrence at the Bronchial Stump Following Lobectomy for Lung Cancer

  • Hwang, Wan Jin;Lee, Yeiwon;Jung, Soo Young;Yeh, Daewook;Park, Soon Hyo;Yoon, Yoo Sang
    • Journal of Chest Surgery
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    • v.49 no.2
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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.

Pancreatic Collision Tumor of Desmoid-Type Fibromatosis and Mucinous Cystic Neoplasm: A Case Report (데스모이드 섬유종증과 점액성 낭성 종양으로 이루어진 췌장의 충돌 종양: 증례 보고)

  • Min Jung Ryu;Jae Woon Kim;Seung Eun Lee;Joon Hyuk Choi
    • Journal of the Korean Society of Radiology
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    • v.82 no.5
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    • pp.1297-1303
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    • 2021
  • Pancreatic collision tumors are rare neoplasm, and cases consisting of ductal adenocarcinoma with a neuroendocrine tumor, intraductal papillary mucinous neoplasm with a neuroendocrine tumor, and solid pseudopapillary neoplasm with a neuroendocrine tumor have been reported. We report a case of a rapidly growing pancreatic collision tumor consisting of desmoid-type fibromatosis and mucinous cystic neoplasm in a 30-year-old pregnant female. To the best of our knowledge, this is the first reported case of a pancreatic collision tumor consisting of desmoid-type fibromatosis and mucinous cystic neoplasm.

Intrathoracic Desmoid Tumor Mimicking Pleural Mass: A Case Report

  • Kim, Na Rae;Chung, Dong-Hae;Lee, Jae-Ik;Jeong, Sung Hwan;Ha, Seung-Yeon
    • Tuberculosis and Respiratory Diseases
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    • v.67 no.5
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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.

CT Findings of Desmoid Tumor arising at Abdominal Wall: Two Cases Report (복벽에 발생한 데스모이드 종양의 급속 조영 CT 소견 : 2례 보고)

  • Cho, Dae-Hyoun;Cho, Jae-Ho;Chang, Jae-Chun
    • Journal of Yeungnam Medical Science
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    • v.12 no.2
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    • pp.386-392
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    • 1995
  • Desmoid tumor is a type of fibromatosis usually arise in deep musculo-aponeurotic structures, primarily of the trunk and extremities. It is characterized by proliferation of fibroblastic tissue and does not metastasize but may be locally aggressive. Eventhough the surgical margin reveals clean, recurrence often occurs. To analyze the extent of the tumor and homodynamic characteristics exactly, we performed IV bolus CT. Desmoid tumors show peripheral rim enhancement on early phase scan and more strong, central enhancement on late phase IV bolus CT, which reflects abundant fibroblastic components of the tumor. We report two cases of pathologically confirmed desmoid tumor performed IV bolus CT.

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Clinical Investigation of Desmoid Tumors (유건종(類腱腫)의 임상적 고찰)

  • Rhee, S.K.;Kim, J.M.;Kim, H.M.;Kang, Y.K.;Kim, Y.S.;Kwon, S.Y.;Lee, K.T.;Kim, I.
    • The Journal of the Korean bone and joint tumor society
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    • v.1 no.2
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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.

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Intrathoracic Desmoid Tumor Presenting as Multiple Lung Nodules 13 Years after Previous Resection of Abdominal Wall Desmoid Tumor

  • Koo, Gun woo;Chung, Sung Jun;Kwak, Joo Hee;Oh, Chang Kyo;Park, Dong Won;Kwak, Hyeon Jung;Moon, Ji-Yong;Kim, Sang-Heon;Sohn, Jang Won;Yoon, Ho Joo;Shin, Dong Ho;Park, Sung Soo;Oh, Young-Ha;Pyo, Ju Yeon;Kim, Tae-Hyung
    • Tuberculosis and Respiratory Diseases
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    • v.78 no.3
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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.

The Imaging Features of Desmoid Tumors: the Usefulness of Diffusion Weighted Imaging to Differentiate between Desmoid and Malignant Soft Tissue Tumors

  • Lee, Seung Baek;Oh, Soon Nam;Choi, Moon Hyung;Rha, Sung Eun;Jung, Seung Eun;Byun, Jae Young
    • Investigative Magnetic Resonance Imaging
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    • v.21 no.3
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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.

A CASE OF AGGRESSIVE FIBROMATOSIS INVADED MANDIBULAR BODY ON THE PAROTID REGION (이하부에 발생한 침습성 섬유종증이 하악체에 침범한 증례 보고)

  • Kim, Young-Jo;Lee, Dong-Keun;Um, In-Woong;Min, Seung-Ki;Chung, Chang-Joo;Kim, Eun-Cheol
    • Maxillofacial Plastic and Reconstructive Surgery
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    • v.16 no.2
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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.

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DESMOPLASTIC FIBROMA OF THE MANDIBLE IN A CHILDREN : A CASE REPORT (어린이의 하악골에 발생한 결합조직형성 섬유종(Desmoplastic fibroma) 1예)

  • Yoon, Jung-Hoon;Lee, Jae-Ho
    • Journal of the korean academy of Pediatric Dentistry
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    • v.28 no.1
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    • pp.171-174
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    • 2001
  • This case describes a case of desmoplastic fibroma of the mandible. A 9-year-old boy was seen with a history of progressive swelling and expansion of the left mandible for one month period. Desmoplastic fibroma was diagnosed on histopathologic examination This report reviews the diagnostic criteria, differential diagnosis and surgical treatment of choice in brief of this uncommon primary bone tumor of the oral and maxillofacial region.

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