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Extra-abdominal Desmoid Tumor

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Topic updated on 03/09/14 10:30pm
  
Introduction
  • A fibrogenic lesion that is the most locally invasive of all benign soft tissue tumors
  • Epidemiology
    • incidence
      • 900 cases a year in US
    • demographics
      • younger individuals (15-40 years of age)
      • slightly female>male
    • location
      • most common shoulder, chest wall/back, thigh
      • >50% are extra-abdominal
      • may have multiple lesions in the same extremity
        •  subsequent mass usually appears more proximally in the same limb
  • Genetics
    • patient's with familial adenomatous polyposis have significant risk for development of extra-abdominal desmoid tumors.  
    • trisomy in chromosome 8 or 20
  • Associated conditions
    • Dupuytren contractures
    • Ledderhose disease
  • Prognosis
    • high risk of recurrence
    • highly unpredictable lesions
    • occasional spontaneous regression
    • no risk of metastasis or malignant transformation, unless related to radiation
Symptoms
  • Presentation
    • usually an enlarging painless mass
  • Physical exam
    • has a distinctive "rock hard" feel on palpation
Imaging
  •  Radiographs
    • play minimal role in diagnosis
  • MRI
    • Indications
      • best means to diagnose
    • findings
      • low signal intensity on T1-weighted images
      • low to medium intensity on T2-weighted images
      • Gadolinium enhances appearance
      • infiltrates muscle
      • 5-10cm in size
      • may erode bone locally
Studies
  • Gross specimen
    • gritty
    • white
    • poorly encapsulated
  • Histology
    • Classic characteristics are 
      • well differentiated fibroblasts 
      • uniform spindle cells with elongated nuclei and occasional mitoses
      • abundant collagen
      • tumor infiltrates adjacent tissues
  • Immunohistochemisty
    • 100% positive for Estrogen receptor-beta  
Treatment
  • Nonoperative
    • low dose-chemotherapy only / tamoxifen 
      • indications
        • inoperable lesions
      • technique
        • tamoxifen works via an estrogen receptor blockage
  • Operative
    • wide surgical resection with radiotherapy
      • indications
        • symptomatic lesion
        • recurrent lesion
          • local recurrence is common (reduced recurrence with radiotherapy)
      • external beam radiation dose- up to 60Gy
IBank
  Location
Xray
Xray
CT
B. Scan
MRI
MRI
Histo(1)

Case A

tibia
 
 
(1) - histology does not always correspond to case 



 

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Qbank (4 Questions)

TAG
(OBQ12.216) A 35-year-old female presents with an enlarging, painless “lump” on her left posterior shoulder. She denies weakness or paresthesias of her left upper extremity. Her maternal uncle, mother, brother, and herself have a history of multiple colonic polyps, jaw osteomas and epidermoid cysts. She ultimately undergoes biopsy of the mass. Which of the following is most likely to be consistent with the histology of the biopsy specimen? Topic Review Topic
FIGURES: A   B   C   D   E  

1. Figure A
2. Figure B
3. Figure C
4. Figure D
5. Figure E

PREFERRED RESPONSE ▶
TAG
(OBQ10.149) Estrogen receptor blockade has been moderately successful treating which of the following tumors, while reducing the morbidity of side-effects associated with previous treatments? Topic Review Topic

1. Desmoid tumor
2. Ewing’s sarcoma
3. Synovial sarcoma
4. Multiple myeloma
5. Giant cell tumor

PREFERRED RESPONSE ▶
TAG
(OBQ09.225) A 20-year-old female presents with a "rock-hard" mass in the calf shown in Figure A. MRI, gross pathology, and histology images are shown in Figure B-D, respectively. Immunohistochemical studies of the mass were strongly positive for Estrogen receptor-beta expression. What is the most likely diagnosis? Topic Review Topic
FIGURES: A   B   C   D    

1. Osteosarcoma
2. Leiomyosarcoma
3. Extraabdominal fibromatoses (desmoid tumor)
4. Angiosarcoma
5. Chondroblastoma

PREFERRED RESPONSE ▶
TAG
(OBQ09.258) Which inherited condition shows a strong association with extra-abdominal desmoid tumors? Topic Review Topic

1. Familial adenomatous polyposis
2. Sinding-Larsen-Johansson syndrome
3. Ollier's disease
4. Fanconi anemia
5. McCune-Albright syndrome

PREFERRED RESPONSE ▶




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