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Desmoid Tumor

Desmoids


Presentation

  • Although rare, it should be included in the differential diagnosis when a patient presents with a large abdominal mass.[ncbi.nlm.nih.gov]
  • A case report and review of the current relevant English literature, carried out using PubMed Medline, are presented.[ncbi.nlm.nih.gov]
  • Consequently, radiotherapy might have worsened an already present malabsorption and so led to steatohepatitis.[ncbi.nlm.nih.gov]
  • CASE REPORT: We herein report a very rare location of sporadic desmoid tumors involving the pancreatic tail, presenting as a cystic lesion. A 51-year-old woman presented with recurrent abdominal pain and weight loss.[ncbi.nlm.nih.gov]
  • CASE PRESENTATION: We present a 27-year-old woman with a paraspinal desmoid tumor, diagnosed 3-years following a motor vehicle accident. Literature is reviewed.[ncbi.nlm.nih.gov]
Weight Loss
  • Herein, we report a 57-year-old woman who presented with recurrent epigastric pain and weight loss. An abdominal computed tomography scan showed an approximately 10-cm mass over the pancreatic head region and dilatation of the pancreatic duct.[ncbi.nlm.nih.gov]
  • A 51-year-old woman presented with recurrent abdominal pain and weight loss.[ncbi.nlm.nih.gov]
Splenectomy
  • Distal pancreatectomy, splenectomy, and debulking surgery were performed.[ncbi.nlm.nih.gov]
Rigor
  • After rigorous physiotherapy, the hip deformity disappeared at 6 months and there was no evidence of recurrence at 2.5-year follow-up, with the patient able to sit cross-legged and squat.[ncbi.nlm.nih.gov]
Abdominal Pain
  • We report a 50-year-old man who presented with the sudden onset of severe abdominal pain. Computerized tomography (CT) demonstrated a huge homogeneous tumor in the lower abdomen that appeared to be in continuity with the distal ileum.[ncbi.nlm.nih.gov]
  • A 20-year-old woman with Gardner syndrome and intra-abdominal desmoid tumors presented with increasing abdominal pain. CT demonstrated a new area of central hypodensity in a presumed desmoid tumor, compressing the left ureter.[ncbi.nlm.nih.gov]
  • We present a case of a 63-year-old woman who complained of abdominal pain, and a contrast-enhanced computed tomography scan revealed a well-circumscribed tumor in the pancreatic tail, measuring 5.1 cm.[ncbi.nlm.nih.gov]
  • A 51-year-old male patient with no history of medical illnesses presented to the emergency department with abdominal pain and a temperature of 39 degrees Celsius. His condition deteriorated rapidly within a few hours.[ncbi.nlm.nih.gov]
  • A 51-year-old woman presented with recurrent abdominal pain and weight loss.[ncbi.nlm.nih.gov]
Abdominal Mass
  • Preoperative computed tomography showed a large left lower abdominal mass. Pathology showed the spindle fibroblastic cell pattern typical of desmoids.[ncbi.nlm.nih.gov]
  • Although rare, it should be included in the differential diagnosis when a patient presents with a large abdominal mass.[ncbi.nlm.nih.gov]
  • A Literature Review. ( 28842766 ) Tzur R...Bogdanov-Berezovsky A 2018 34 Screening-detected desmoid tumor of the breast: findings at conventional imaging and digital breast tomosynthesis. ( 29375894 ) Samardzic T...Skaane P 2018 35 New intra-abdominal[malacards.org]
Severe Abdominal Pain
  • We report a 50-year-old man who presented with the sudden onset of severe abdominal pain. Computerized tomography (CT) demonstrated a huge homogeneous tumor in the lower abdomen that appeared to be in continuity with the distal ileum.[ncbi.nlm.nih.gov]
  • Other symptoms include: rectal bleeding and severe abdominal pain, when a desmoid tumor in the abdomen places pressure on the intestines localized pain or stiffness, when a desmoid tumor compresses nerves, blood vessels, or an organ limping and other[medicalnewstoday.com]
Epigastric Pain
  • Three years later, he presented with acute epigastric pain and obstructive ileum. While a mass in the left lateral abdominal region was palpated. The patient underwent laparotomy, some adhesions were dissected and biopsies were taken from the mass.[ncbi.nlm.nih.gov]
  • Herein, we report a 57-year-old woman who presented with recurrent epigastric pain and weight loss. An abdominal computed tomography scan showed an approximately 10-cm mass over the pancreatic head region and dilatation of the pancreatic duct.[ncbi.nlm.nih.gov]
Vomiting
  • A 43-year-old woman presented with headaches, dizziness, equilibrium disorders, nausea, and vomiting due to a hemangioblastoma in posterior fossa. A gross total resection was achieved.[ncbi.nlm.nih.gov]
  • 49) 0 14 (39) 1 (3) Myalgia 18 (37) 1 (2) 12 (33) 0 Alopecia 18 (37) 0 3 (8) 0 Arthralgia 17 (35) 1 (2) 9 (25) 0 Abdominal pain 15 (31) 1 (2) 9 (25) 4 (11) Anorexia 15 (31) 0 9 (25) 0 Constipation 11 (22) 0 4 (11) 0 Oral mucositis 11 (22) 0 6 (17) 0 Vomiting[nejm.org]
Thrombosis
  • This report underlines the potential of imaging investigations of abdomen and vena cava if pulmonary embolism is suspected, especially when there is no evidence of peripheral venous thrombosis or other predisposing factors.[ncbi.nlm.nih.gov]
Flexion Contracture
  • The authors present a rare case of a hip contracture in a 28-year-old active man who presented with a functionally disabling hip flexion contracture of 20 with further flexion possible up to 130 , a 45 abduction contracture, and a 20 external rotation[ncbi.nlm.nih.gov]
Adnexal Mass
  • She was diagnosed as a case of adnexal mass with mild hydroureter & mild hydronephrosis and it was decided to operate on the patient. The surgical approach was transabdominal.[ncbi.nlm.nih.gov]
Dizziness
  • A 43-year-old woman presented with headaches, dizziness, equilibrium disorders, nausea, and vomiting due to a hemangioblastoma in posterior fossa. A gross total resection was achieved.[ncbi.nlm.nih.gov]

Workup

Atelectasis
  • […] rapid, especially for DTs occurring in anatomically critical compartments, including the thoracic cavity.The authors report a case of a 71-year-old man with a known mediastinal mass incidentally detected 4 months ago, presenting dyspnea with right-sided atelectasis[ncbi.nlm.nih.gov]

Treatment

  • Because of the variable nature of the disease and the potential morbidity of treatment, some cases of DTF may do better without treatment; simple observation is often the best initial treatment.[ncbi.nlm.nih.gov]
  • The tumor grew bigger and bigger during a treatment period. Finally, hyperthermia treatment was applied to the tumor. The size of the recurrent desmoid tumor reduced 75% by hyperthermia treatment for 46-month.[ncbi.nlm.nih.gov]
  • Treatment starts with a wait-and-see policy, however, more aggressive treatments like broad margin resection surgery might be necessary in case of tumor progression.[ncbi.nlm.nih.gov]
  • Most patients require 2-4 yearly treatments to control the tumor.[web.archive.org]
  • At present there is no definite and effective method of treatment.[ncbi.nlm.nih.gov]

Prognosis

  • We report the case for its rarity and huge size and to emphasize a regular follow-up because the long-term prognosis is currently unknown.[ncbi.nlm.nih.gov]
  • However, early detection of the tumor play an important role, MRI is now the most important method for the detection of tumor extent, which facilitates the treatment choice as well as the prediction of prognosis.[ncbi.nlm.nih.gov]
  • The outcome of this case study is encouraging, and long-term follow-up studies are required to establish the effect of treatment with celecoxib on the prognosis of DTs.[ncbi.nlm.nih.gov]
  • What is the prognosis for someone with a desmoid tumor? The estimate of how a disease will affect you long-term is called prognosis.[cancer.gov]
  • Abstract Desmoid tumors are rare soft tissue tumors with limited data on their management and prognosis.[ncbi.nlm.nih.gov]

Etiology

  • Aggressive fibromatosis or desmoid tumor, is a histologically benign entity with unknown etiology that may present a serious clinical course.[ncbi.nlm.nih.gov]
  • The etiology is still uncertain. Although rare, this distinctive tumor should be kept in mind in the differential diagnosis of palpable masses and especially those occurring around the previous skin incisions.[ncbi.nlm.nih.gov]
  • The etiology of desmoids has not been determined. We report the case of anterior abdominal wall desmoid tumor in a female patient with previous history of cesarean section.[ncbi.nlm.nih.gov]
  • The etiology of desmoid tumors are uncertain but may be related to operation, trauma or hormonal factors. The authors report a 17-year old woman, gravida 1, para 1 with a mass at her lower abdominal wall during the fifth month of gestation.[ncbi.nlm.nih.gov]
  • The etiological factors suggested include genetic, endocrine and physical factors. About fifty percent of desmoid tumors arise in the abdominal region.[ncbi.nlm.nih.gov]

Epidemiology

  • Epidemiology Although we don’t yet know the cause of these tumors, researchers have found that they tend to occur in children who have a family history of polyposis coli, a hereditary colon cancer.[curesarcoma.org]
  • Division of Epidemiology Imperial College London UK 3. Polyposis Registry St. Mark’s Hospital & Imperial College London UK[doi.org]
  • Hui, Epidemiology and Etiology of Sarcomas, Surgical Clinics of North America, 10.1016/j.suc.2016.05.005, 96, 5, (901-914), (2016). M. Creuzé, P. Afchain, A. Munck, J. Viala, A. Bonnard and V.[doi.org]
  • The tumors regress during menopause [10] and after tamoxifen treatment. [11] Desmoid tumors may regress after exposure to oral contraceptives. [12] The proliferative response of fibroblasts to estrogen has been established. [13] Epidemiology Frequency[emedicine.medscape.com]
  • Journal of clinical epidemiology 54, 343–349 (2001). 74. Xi, B., Gu, H., Baniasadi, H. & Raftery, D. Statistical analysis and modeling of mass spectrometry-based metabolomics data. Methods in molecular biology (Clifton, N.[nature.com]
Sex distribution
Age distribution

Pathophysiology

  • This review used a PubMed search from January 1, 1980, through October 31, 2016, using the terms fibromatosis and desmoid and discusses DTF disease characteristics, pathophysiology, and treatment options as well as examines several cases illustrating[ncbi.nlm.nih.gov]
  • In FAP: majority (51 - 67%) are intraabdominal or in the abdominal wall Sporadic: extraabdominal are more common Tendency of premenopausal and pregnant women to develop fibromatosis in the abdominal wall musculoaponeurosis ( Fam Cancer 2006;5:191 ) Pathophysiology[pathologyoutlines.com]
  • Pathophysiology Although desmoid tumors most commonly arise from the rectus abdominis muscle in postpartum women and in scars due to abdominal surgery, they may arise in any skeletal muscle.[emedicine.medscape.com]

Prevention

  • Desmoid tumors tend to be locally infiltrative, therefore, the fields must be generous to prevent marginal recurrence. The radiation dose appropriate for treating desmoid tumors remains controversial.[ncbi.nlm.nih.gov]
  • No treatment was given postoperatively to prevent desmoid tumor recurrence. After a 1-year follow-up, no recurrence was observed.[ncbi.nlm.nih.gov]
  • Protecting this progenitor cell population might prevent tumor formation in patients harboring a germline APC mutation, where fibromatosis is currently the leading cause of mortality. 2010 AACR. [Indexed for MEDLINE] Free full text[ncbi.nlm.nih.gov]
  • Types of treatment currently being studied include: Angiogenesis inhibitors - These substances may be able to prevent the growth of tumors by blocking the formation of new blood vessels that feed the tumors.[childrenshospital.org]
  • For now, however, no data suggest that any specific intervention can prevent desmoid tumors. Prognosis and survival rate Desmoid tumors are so rare that the National Cancer Institute do not have access to an accurate survival rate.[medicalnewstoday.com]

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