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1. Levy AD, Manning MA, Miettinen MM: Soft-Tissue Sarcomas of the Abdomen and Pelvis: Radiologic-Pathologic Features, Part 2-Uncommon Sarcomas. Radiographics; 2017 May-Jun;37(3):797-812
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Soft-Tissue Sarcomas of the Abdomen and Pelvis: Radiologic-Pathologic Features, Part 2-Uncommon Sarcomas.
  • Soft-tissue sarcomas occurring in the abdomen and pelvis are an uncommon but important group of malignancies.
  • Recent changes to the World Health Organization classification of soft-tissue tumors include the movement of gastrointestinal stromal tumors (GISTs) into the soft-tissue tumor classification.
  • Liposarcoma is the most common retroperitoneal sarcoma, and leiomyosarcoma is the second most common.
  • GIST, liposarcoma, and leiomyosarcoma account for the majority of sarcomas encountered in the abdomen and pelvis and are discussed in part 1 of this article.
  • Undifferentiated pleomorphic sarcoma (previously called malignant fibrous histiocytoma), dermatofibrosarcoma protuberans, solitary fibrous tumor, malignant peripheral nerve sheath tumor, rhabdomyosarcoma, extraskeletal chondro-osseous sarcomas, vascular sarcomas, and sarcomas of uncertain differentiation uncommonly arise in the abdomen and pelvis and the abdominal wall.
  • The anatomic location and clinical history are important factors in the differential diagnosis of these lesions because metastasis, more-common sarcomas, borderline fibroblastic proliferations (such as desmoid tumors), and endometriosis have imaging findings that overlap with those of these uncommon sarcomas.
  • In this article, the clinical, pathologic, and imaging findings of uncommon soft-tissue sarcomas of the abdomen and pelvis and the abdominal wall are reviewed, with an emphasis on their differential diagnosis.
  • [MeSH-major] Abdomen / diagnostic imaging. Abdomen / pathology. Pelvis / diagnostic imaging. Pelvis / pathology. Sarcoma / diagnostic imaging. Sarcoma / pathology
  • [MeSH-minor] Diagnosis, Differential. Humans

  • MedlinePlus Health Information. consumer health - Soft Tissue Sarcoma.
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  • (PMID = 28493803.001).
  • [ISSN] 1527-1323
  • [Journal-full-title] Radiographics : a review publication of the Radiological Society of North America, Inc
  • [ISO-abbreviation] Radiographics
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
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2. Kasper B, Ströbel P, Hohenberger P: Desmoid tumors: clinical features and treatment options for advanced disease. Oncologist; 2011;16(5):682-93
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  • [Title] Desmoid tumors: clinical features and treatment options for advanced disease.
  • Desmoid tumors describe a rare monoclonal, fibroblastic proliferation characterized by a variable and often unpredictable clinical course.
  • Proof of a CTNNB1 mutation may be useful when the pathological differential diagnosis is difficult and location might be predictive for disease recurrence.
  • Because of the heterogeneity of the biological behavior of desmoids, including long periods of stable disease or even spontaneous regression, treatment needs to be individualized to optimize local tumor control and preserve patients' quality of life.
  • This review describes treatment options and management strategies for patients with desmoid tumors with a focus on advanced disease.
  • [MeSH-major] Fibromatosis, Aggressive / pathology. Fibromatosis, Aggressive / therapy
  • [MeSH-minor] Adenomatous Polyposis Coli Protein / genetics. Combined Modality Therapy. DNA Mutational Analysis. Diagnosis, Differential. Fibromatosis, Abdominal / pathology. Fibromatosis, Abdominal / therapy. Humans. Mesentery / pathology. Neoplasm Staging. beta Catenin / genetics

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  • (PMID = 21478276.001).
  • [ISSN] 1549-490X
  • [Journal-full-title] The oncologist
  • [ISO-abbreviation] Oncologist
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / APC protein, human; 0 / Adenomatous Polyposis Coli Protein; 0 / beta Catenin
  • [Other-IDs] NLM/ PMC3228186
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3. Montgomery C, Emory C, Adams S, Cohen J, Pitcher JD, Potter BK, Temple HT: Treatment of extra - abdominal desmoid tumors with chemotherapy. Cancers (Basel); 2011;3(3):3394-404
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  • [Title] Treatment of extra - abdominal desmoid tumors with chemotherapy.
  • Fibromatosis, or extra-abdominal desmoid tumor, is a benign disease which often has an aggressive clinical course that can be difficult to treat.
  • We performed a retrospective review of 16 patients (12 females and four males) with a mean age of 34.2 years treated with methotrexate and vinblastine for newly diagnosed or recurrent extra-abdominal desmoid tumor.
  • The mean age of our patient cohort was 34.2 years (range 11-70), and the mean tumor size was 11.5 cm (range 2.5-21.2 cm).
  • Eight of 14 patients demonstrated a radiologic decrease in tumor size.
  • The use of methotrexate and vinblastine/vinorelbine in the management of fibromatosis appears to be an effective treatment with minimal treatment-related side effects.

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  • (PMID = 24212959.001).
  • [ISSN] 2072-6694
  • [Journal-full-title] Cancers
  • [ISO-abbreviation] Cancers (Basel)
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Switzerland
  • [Other-IDs] NLM/ PMC3759201
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4. Pinheiro LV, Fagundes JJ, Coy CS, Cabello C, Toro I, Michellino M, Fachina PH, Ward M, Leal RF, Ayrizono Mde L: Multiple desmoid tumors in a patient with Gardner's syndrome - Report of a case. Int J Surg Case Rep; 2014;5(7):370-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Multiple desmoid tumors in a patient with Gardner's syndrome - Report of a case.
  • INTRODUCTION: Desmoid tumor (DT) is a common manifestation of Gardner's Syndrome (GS), although it is a rare condition in the general population.
  • DT in patients with GS is usually located in the abdominal wall and/or intra-abdominal cavity.
  • PRESENTATION OF CASE: We report a case of a 32 years-old female patient with familial adenomatous polyposis (FAP), who was already submitted to total colectomy and developed multiple DT, located in the abdominal wall and in the left breast.
  • Wide surgical resections of the left breast and the abdominal wall tumors were performed in separate steps.
  • Polypropylene mesh reconstruction and muscle flaps were needed to cover the defects of the thoracic and abdominal walls.
  • After partial necrosis of the adipose-cutaneous flap in the abdomen that required a new skin graft, she had a satisfactory outcome with complete healing of the surgical incisions.
  • GS patients must be followed up closely, and clinical examination, associated with imaging studies, should be performed to detect any signs of tumor.

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  • [Copyright] Copyright © 2014 The Authors. Published by Elsevier Ltd.. All rights reserved.
  • (PMID = 24858982.001).
  • [ISSN] 2210-2612
  • [Journal-full-title] International journal of surgery case reports
  • [ISO-abbreviation] Int J Surg Case Rep
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Netherlands
  • [Other-IDs] NLM/ PMC4064399
  • [Keywords] NOTNLM ; Desmoid tumor / Familial adenomatous polyposis / Gardner's syndrome / Surgical approach
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5. Kujak JL, Liu PT, Johnson GB, Callstrom MR: Early experience with percutaneous cryoablation of extra-abdominal desmoid tumors. Skeletal Radiol; 2010 Feb;39(2):175-82
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Early experience with percutaneous cryoablation of extra-abdominal desmoid tumors.
  • OBJECTIVE: Surgical resection, radiation therapy and chemotherapy are all accepted as standard treatments for extra-abdominal desmoid (EAD) tumors, but their effectiveness has been limited by frequent local recurrence.
  • The purpose of this article is to describe our early experiences with using percutaneous cryoablation for local control of extra-abdominal desmoid tumors in five patients whose tumors had failed to respond to standard therapy.
  • Three of these patients had been referred for cryoablation for local tumor control, and two had been referred for palliation of inoperable tumors.
  • RESULTS: For the three patients referred for local control of EAD tumors, complete tumor coverage with the ablation zones was achieved.
  • The third patient, with a 6.1 cm mass, reported improved mild pain at 6 months, and imaging showed a moderate decrease of tumor size.
  • For the two patients referred for palliative therapy, initial partial pain relief was felt 2 weeks after the procedure, At long-term (58 months) follow-up of one patient with a 9.1 cm mass, the tumor was still present although reduced in size, and local pain had returned to its former moderate level.
  • [MeSH-major] Cryosurgery / methods. Fibromatosis, Aggressive / diagnosis. Fibromatosis, Aggressive / surgery
  • [MeSH-minor] Abdominal Neoplasms / diagnosis. Abdominal Neoplasms / surgery. Adolescent. Adult. Child. Female. Humans. Male. Pilot Projects. Treatment Outcome. Young Adult

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  • (PMID = 19768644.001).
  • [ISSN] 1432-2161
  • [Journal-full-title] Skeletal radiology
  • [ISO-abbreviation] Skeletal Radiol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article
  • [Publication-country] Germany
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6. Majors J, Stoikes NF, Nejati R, Deneve JL: Resection and Abdominal Wall Reconstruction of a Desmoid Tumor with Endometrioma Features. Case Rep Surg; 2016;2016:9453450
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Resection and Abdominal Wall Reconstruction of a Desmoid Tumor with Endometrioma Features.
  • Desmoid tumors are rare, musculoaponeurotic mesenchymal origin tumors arising from the proliferation of well-differentiated fibroblasts.
  • Desmoid tumors may arise from any location with the abdominal cavity, abdominal wall and extremity locations being most frequent.
  • We present the case of a 35-year-old female with a history of endometriosis who presented palpable abdominal mass and cyclic abdominal pain.
  • Resection was performed for a presumed desmoid soft tissue tumor.
  • Final pathology demonstrated desmoid histology admixed with abdominal wall endometriosis (endometrioma).
  • This unique pathologic finding has only been rarely reported and is discussed with a brief review of the literature.

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  • (PMID = 27247824.001).
  • [ISSN] 2090-6900
  • [Journal-full-title] Case reports in surgery
  • [ISO-abbreviation] Case Rep Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Other-IDs] NLM/ PMC4877470
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7. Fleetwood VA, Zielsdorf S, Eswaran S, Jakate S, Chan EY: Intra-abdominal desmoid tumor after liver transplantation: A case report. World J Transplant; 2014 Jun 24;4(2):148-52
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Intra-abdominal desmoid tumor after liver transplantation: A case report.
  • We are reporting the first documented case of an abdominal desmoid tumor presenting primarily after liver transplantation.
  • This tumor, well described in the literature as occurring both in conjunction with familial adenomatous polyposis as well as in the post-surgical patient, has never been noted after solid organ transplantation and was therefore not included in our differential upon presentation.
  • Definitive diagnosis required the patient to undergo surgical excision and immunochemical staining of the mass for confirmation.
  • In a population of patients who received a small bowel transplant after they developed short gut post radical resection of aggressive fibromatosis, only rare recurrences were seen.
  • No connection of tumor development with immunosuppression or need to decrease immunosuppressant treatment has been demonstrated in these patients.
  • Our case and the literature show the risk of this tumor presenting in the post-transplantation patient and the need for a high index of suspicion in patients who present with a complex mass after transplantation to prevent progression of the disease beyond a resectable lesion.

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  • (PMID = 25032104.001).
  • [ISSN] 2220-3230
  • [Journal-full-title] World journal of transplantation
  • [ISO-abbreviation] World J Transplant
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Other-IDs] NLM/ PMC4094950
  • [Keywords] NOTNLM ; Desmoid / Immunosuppression / Intra-abdominal fibromatosis / Liver transplantation / Recurrence / Solid organ transplantation
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8. Tan CH, Pua U, Liau KH, Lee HY: Mesenteric desmoid tumour masquerading as a fat-containing cystic mass. Br J Radiol; 2010 Oct;83(994):e200-3
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Mesenteric desmoid tumour masquerading as a fat-containing cystic mass.
  • Mesenteric desmoid tumour (MDT) is an uncommon neoplasm that typically presents as a solid soft-tissue mass on cross-sectional imaging.
  • [MeSH-major] Cysts / radiography. Fibromatosis, Abdominal / radiography. Mesenteric Cyst / radiography
  • [MeSH-minor] Abdominal Pain / etiology. Diagnosis, Differential. Female. Humans. Middle Aged. Tomography, X-Ray Computed. Vomiting / etiology

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  • (PMID = 20846976.001).
  • [ISSN] 1748-880X
  • [Journal-full-title] The British journal of radiology
  • [ISO-abbreviation] Br J Radiol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC3473748
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9. Zeng WG, Zhou ZX, Liang JW, Hou HR, Wang Z, Zhou HT, Zhang XM, Hu JJ: Prognostic factors for desmoid tumor: a surgical series of 233 patients at a single institution. Tumour Biol; 2014 Aug;35(8):7513-21
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Prognostic factors for desmoid tumor: a surgical series of 233 patients at a single institution.
  • Desmoid tumors are rare soft tissue tumors with limited data on their management and prognosis.
  • We sought to determine the rates of recurrence after surgery for desmoid tumors and analyze factors predictive of recurrence-free survival (RFS).
  • From February 1976 to October 2011, 233 consecutive patients with desmoid tumors who underwent macroscopically complete resection were included in this study.
  • Patterns of presentation included primary (n = 156, 67.0 %) and locally recurrent (n = 77, 33.0 %) disease initially treated elsewhere.
  • Median follow-up was 54 months; recurrence disease was observed in 62 (26.6 %) patients.
  • Factors associated with worse RFS were tumor size larger than 5 cm (hazard ratio (HR) = 3.757; 95 % CI, 1.945-7.259; p < 0.001), extra-abdominal tumor location (abdominal wall referent; HR = 3.373; 95 % CI, 1.425-7.984; p = 0.006), and R1 resection status (HR = 1.901; 95 % CI, 1.140-3.171; p = 0.014).
  • Regardless of primary or recurrent disease, surgical resection remains central to the management of patients with desmoid tumors.
  • [MeSH-major] Fibromatosis, Aggressive / mortality

  • Genetic Alliance. consumer health - Desmoid Tumor.
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  • (PMID = 24789435.001).
  • [ISSN] 1423-0380
  • [Journal-full-title] Tumour biology : the journal of the International Society for Oncodevelopmental Biology and Medicine
  • [ISO-abbreviation] Tumour Biol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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10. Monneur A, Chetaille B, Perrot D, Guiramand J, Bertucci F: Dramatic and delayed response to Doxorubicin-dacarbazine chemotherapy of a giant desmoid tumor: case report and literature review. Case Rep Oncol; 2013 Jan;6(1):127-33
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Dramatic and delayed response to Doxorubicin-dacarbazine chemotherapy of a giant desmoid tumor: case report and literature review.
  • Desmoid tumors are benign, slow-growing mesenchymal tumors.
  • The treatment is challenging, particularly in the case of huge intra-abdominal locations.
  • We, herein, report on a 21-year-old patient with a giant intra-abdominal desmoid tumor occupying substantially the entire abdominal cavity.
  • After 6 cycles, the computed tomography scan showed a partial response (regression of tumor volume by 55%).
  • During follow-up, the tumor continued to regress: 25 months after the end of chemotherapy, the tumor volume had regressed by 95% when compared to the start of computed tomography and by 90% when compared to the end of chemotherapy.
  • Thirty-three months after the diagnosis, the patient is alive without any symptom.
  • We review the literature and discuss the challenging issue regarding treatment of desmoid tumors.

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  • (PMID = 23569447.001).
  • [ISSN] 1662-6575
  • [Journal-full-title] Case reports in oncology
  • [ISO-abbreviation] Case Rep Oncol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Switzerland
  • [Other-IDs] NLM/ PMC3618027
  • [Keywords] NOTNLM ; Chemotherapy / Dacarbazine / Desmoid tumor / Doxorubicin
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