Extra-abdominal Desmoid Tumor Research Articles

Subsequently Occurring Leiomyosarcoma and Desmoid Tumor of the Right Thigh

We present the case of a 44-year-old patient with a primary leiomyosarcoma of the right upper thigh, who developed a second mesenchymal lesion at the same thigh, diagnosed as an extra-abdominal desmoid tumor, some months after surgical treatment of his leiomyosarcoma. Clinically, histomorphologically, DNA cytometrically, immunohistochemically and radiologically the different histogenetic origin of the two lesions could be proven. The patient died 17 months after his first tumor diagnosis of lung metastases of his leiomyosarcoma.

Desmoplastic fibroma of bone

Desmoplastic fibroma is a rare primary tumor of bone that histologically and biologically mimics the extra-abdominal desmoid tumor of soft tissue. This study reviews 27 cases of desmoplastic fibroma, consisting of 9 from the Mayo Clinic files and 18 from our consultation files. There was a male predominance, and 74% of the patients were in the first 3 decades of life. The most frequent sites of involvement were the metaphysis of long bones and the mandible. Radiographically, the tumors were lucent, expansile lesions with well-defined margins. Histologically, they contained slender spindle cells and various amounts of collagen fibers. En bloc resection is the treatment of choice because a high incidence of recurrence was noticed after lesional curettage.

Extra-abdominal desmoid tumors

A follow-up study of 53 patients with extra-abdominal desmoid tumor is presented. Fine-needle aspiration biopsy seemed to be valuable in the differentiation between a desmoid tumor and a truly malignant soft tissue tumor. The angiographic pictures did not differ from those of other soft tissue tumors, but valuable topographic information was obtained from the angiography in the preoperative planning. The overall local recurrence rate was 17/44, and 15 of the recurrences occurred within the first 2 years. Complete removal of the affected muscle or muscle group is recommended. Local control can be achieved by non-ablative surgery even after multiple recurrences. Only one out of 53 patients died of the tumor disease. Radiotherapy can be attempted in extensive tumors, and amputation should be reserved for tumors which have failed to respond to this treatment.

けい部に生じたＥｘｔｒａ‐ａｂｄｏｍｉｎａｌ ｄｅｓｍｏｉｄ ｔｕｍｏｒの１例

Extra-abdominal desmoid tumor is a tumor which occurs from the musculo-aponeurotic structures and had a tendency to invade the neighboring soft tissue or bone.Recently we have treated a case of this kind of tumor of the neck.A 15-year-old male complained of a swelling on the left side of his neck for about 2 years. He visited another hospital where his tumor was resected. He had a recurrence six months later, so he was admitted to our hospital. We performed the resection of the tumor, and the pathological diagnosis of the tumor was fibromatosis. At 8 months later after the operation, there was no evidence of recurrence.

The desmoid tumor. II. Analysis of factors possibly contributing to the etiology and growth behavior.

Eighty histologically verified cases of the desmoid tumor (DT) have been analyzed with regard to factors possibly contributing to the etiology and/or growth behavior of this uncommon neoplasm. Considering the four statistical age components, the "fertile" female and "menopausal" varieties of the DT grew distinctly faster (0.1 less than P greater than 0.05 and less than 0.05), and the female "juvenile" variety distinctly slower (0.1 less than P greater than 0.05) than the male DTs. In a visual estimate, the fertile female patients had a significant (P less than 0.01) predisposition to estrogen predominance, while fewer patients than expected displayed progesterone predominance or were at balance (P = NS and less than 0.01, respectively). Thirty-two per cent of the patients with an abdominal DT had been previously operated in the region of subsequent tumor growth. Significantly more pregnancies were observed in patients with abdominal DT than with extra-abdominal DT (P less than 0.05). On only one occasion did sigmoideoscopy reveal colonic polyposis (Gardner's syndrome). The most striking observation was, however, that up to 80% of the affected patients (compared with less than 5% in the normal control population, P less than 0.05) had multiple minor bone anomalies demonstrable by x-ray screening of the mandible, chest, and long bones. We suggest that a generalized (inherited or mutant) defect in growth regulation of connective tissue is the most important underlying cause for the DT. However, the other factors, including hormonal effects, trauma, and pregnancy contribute to the growth behavior of the tumor.

The desmoid tumor. I. Incidence, sex-, age- and anatomical distribution in the Finnish population.

We have (1) estimated the incidence of desmoid tumor (DT) in the Finnish population, and (2) defined statistically four major age components of the DT with different biological properties. The incidence of the DT, based on admissions to four separate hospitals and on the number of pathological biopsy specimens analyzed at the Central Pathological Laboratory of Helsinki University, is 2.4-4.3 new cases per 10(6) inhabitants per annum. Statistical analysis demonstrated four major age components where the site of the tumor and/or sex of the patient were non-randomly distributed: "juvenile" DT, a predominantly extra-abdominal desmoid tumor of the female sex; "fertile" DT, a nearly exclusively abdominal DT of fertile females; "menopausal" DT, a predominantly abdominal tumor where the sex ratio approaches one:one; and "senescent" DT, where abdominal and extra-abdominal varieties are equally frequently encountered and where the sex ratio of the affected patients is one:one.

Aggressive Fibromatosis of the Tongue

Fibromatoses are uncommon, locally aggressive lesions that may involve the structures of the head and neck. They are most commonly found in and along the fascial planes of the neck muscles but can involve other structures, including the oral cavity, nose, nasopharynx or paranasal sinuses. An unusual case of an extra-abdominal desmoid tumor is presented and a review of the anatomical, clinical and pathological features of these tumors is discussed.

Extra-abdominal desmoid of the axillary tail mimicking breast carcinoma

A case is reported of an extra-abdominal desmoid tumor presenting as an axillary tail breast mass. This represents the third reported case and the first description of the roentgenographic findings of this entity. That this tumor can be confused with carcinoma of the breast is emphasized.