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.
Abdominal Wall ; Extremities ; Fibroblasts ; Fibroma ; Fibromatosis, Aggressive* ; Recurrence

Fibromatoses comprise many different entities of well-differentiated fibroblastic proliferation with variable collagen production and form a firm nodular mass. Abdominal fibromatosis is distinguishable from other forms of fibromatosis because of its location and its tendency to occur in women of childbearing age during or following pregnancy. Abdominal fibromatosis in children is an extremely rare condition. A 15-month-old boy presented with an abdominal wall mass that had recently increased in size. Mass excision was perfomed. The tumor was 4.3x4.1 cm and partly circumscribed. Histologically, the tumor was composed of parallel long fascicles of spindle-cells with a uniform appearance. The edges of the resected mass were infiltrative, and the surgical margins were positive. Mitotic figures were <1/10 high power fields. No cellular atypia or necrosis was present. The tumor cells were positive for vimentin and nuclear beta-catenin staining.
Abdominal Wall ; beta Catenin ; Child* ; Collagen ; Female ; Fibroblasts ; Fibroma ; Fibromatosis, Abdominal* ; Humans ; Infant ; Necrosis ; Pregnancy ; Vimentin

PURPOSE: Laparoscopic approaches to the anterior abdominal wall are difficult because of the inherent limitations of laparoscopic surgery. METHODS: A 25-year-old young female visited to our hospital with an incidentally-found palpable abdominal mass. Computed tomography revealed a 7-cm round enhancing mass arising from the left posterior rectus sheath. The patient underwent laparoscopic excision of the inner mass arising from the anterior abdominal wall. RESULTS: The operation lasted for 45 minutes and there was no measurable bleeding during the procedure. Pathological assessment revealed that the tumor was a DTF 7.0 cm in size. The patient was discharged on the second postoperative day in good condition, and is currently being followed on a routine basis for surveillance without adjuvant therapy. CONCLUSION: Laparoscopic approach for the anterior abdominal wall tumor could be feasible in select patients. This minimally-invasive approach helps to ensure good cosmetic outcomes and quality of life.
Abdominal Wall* ; Adult ; Female ; Fibroma* ; Fibromatosis, Abdominal ; Hemorrhage ; Humans ; Laparoscopy ; Quality of Life

Female ; Fibromatosis, Abdominal ; diagnostic imaging ; pathology ; surgery ; Humans ; Middle Aged ; Radiography ; Rupture, Spontaneous ; surgery

The fibromatosis is a broad group of benign fibrous tissue proliferations of similar microscopic appearance that are intermediate in their biological behavior between benign fibrous lesions and fibrosarcoma. Although various series have been reported of abdominal wall and extra-abdominal desmoid tumors, intra-abdominal desoids are extremely rare. We experienced a case with mesenteric fibroma-tosis occuring in a 30 year-old male. He was admitted to the Kangbuk Samsung hospital complaining of right lower quadrant abdominal mass and abdominal bloating sense. Utrasonography and computed tomography of the abdomen showed a solid mass in the left abdomen surrounded by loops of small bowel. At explorative laparotomy, there was a hard, well circumscribed round mass (25 X 15 X 12 cm) in the mesentery of the terminal ilem. After the tumor was dissected from the retro-peritoneum and surrounding tissues, segmental re- section of ileum with end-to-end anastomosis was performed. On the histopathologic examination, it was confirmed as mesenteric fibromatosis. A brief review of the literature on mesentery fibromatosis was done.
Abdomen ; Abdominal Wall ; Adult ; Fibroma* ; Fibromatosis, Aggressive ; Fibrosarcoma ; Humans ; Ileum ; Laparotomy ; Male ; Mesentery

Mesenteric fibromatosis is a monoclonal, fibroblastic proliferation arising from musculoaponeurotic structure, and it is distinctive lesions defined as a group of non-metastasizing fibroblastic tumors which has local invasion and has a high recurrence rate after the surgical excision. The main treatment modality is the surgical excision. Radiation therapy, chemotherapy, and hormone therapy are also known as useful treatments. We report our experience of a recent case of Mesenteric fibromatosis. A 62-year old female patient had undergone gastrectomy due to gastric cancer. 18 months after gastrectomy, we detected an abdominal mass. The preoperative radiologic findings were suggestive of recurrence. Exploratory laparotomy was performed and post-operative pathologic diagnosis was confirmed as fibromatosis. We report a patient with mesenteric fibromatosis that mimic recurrence after gastrectomy for gastric cancer.
Female ; Fibroblasts ; Fibroma ; Fibromatosis, Abdominal ; Gastrectomy ; Humans ; Hydrazines ; Laparotomy ; Recurrence ; Stomach Neoplasms

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.
Abdominal Wall* ; Fibromatosis, Aggressive* ; Lung* ; Multiple Pulmonary Nodules ; Neoplasm Metastasis ; Pleura ; Thoracic Wall ; Thorax

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 × 10⁻⁶ mm²/s) was significantly higher than the mean of the malignant soft tissue tumors (873 × 10⁻⁶ mm²/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.
Abdominal Wall ; Diffusion* ; Electrons ; Fibroma ; Fibromatosis, Aggressive* ; Hemorrhage ; Humans ; Magnetic Resonance Imaging

PURPOSE: This research was conducted to assess the incidence, clinical characteristics, and treatment outcomes for desmoid tumors in patients with familial adenomatous polyposis (FAP). METHODS: At Medical Center, we recruited 47 patients who had been diagnosed as having intraabdominal or abdominal wall desmoid tumor between Aug. 1995 and Dec. 2005. We compared FAP-associated desmoid tumors with non-FAP-associated desmoid tumors according to clinical characteristics and treatment outcomes. RESULTS: Desmoid tumors developed 12/46 (26.1%) in FAP, 1/14 (7.1%) in attenuated FAP and 34 in non-FAP associated. Unlike non-FAP-associated desmoid tumors, the occurrence of FAP-associated desmoid tumors in tended to be higher in the earlier age groups (< or =40 yrs, 92.3% vs 67.6%, P=0.082) and no sexual predominancy was observed (male:female ratio of 1.2:1 vs a tumor ratio 1:3.9, P=0.033). Intraabdominal-type desmoid tumors associated for the majority of FAP-associated desmoid tumors (92.3% vs 38.2%, P=0.002), and 70% of the desmoid tumors occurred within 3 years after total proctocolectomy. In the treatment of FAP-associated intraabdominal desmoid tumors, surgery was performed in 7 cases (58.3%), and complete resections were done in only 3 cases (25%), with one recurrence. In non-FAP-associated desmoid tumors, complete resection was possible in 10 cases (76.9%), and there was no recurrence (P=0.036). The medical treatment for unresectable or incompletely resectable cases in cases of non-FAP-associated desmoid tumor was good, but for FAP-associated desmoid tumors, the effectiveness was not good, and further investigation was needed. CONCLUSIONS: Intraabdominal desmoid tumors in FAP patients occurred frequently in the early (< or =3 yrs) postoperative period, and the treatment, outcome including surgery and medication, outcome was not good in patients with FAP-associated desmoid tumors.
Abdominal Wall ; Adenomatous Polyposis Coli ; Fibromatosis, Aggressive ; Humans ; Incidence ; Postoperative Period ; Recurrence

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.
Abdomen ; Diagnosis, Differential ; Fibromatosis, Abdominal ; Fibromatosis, Aggressive ; Follow-Up Studies ; Granuloma, Plasma Cell ; Humans ; Mesothelioma ; Pleura ; Pleural Cavity ; Solitary Fibrous Tumors ; Vimentin