Extra-abdominal Desmoid Tumor Research Articles

Full-term gestation and transvaginal delivery after wide resection of an abdominal desmoid tumor during pregnancy

A 29-year-old female was diagnosed with a symptomatic, extra-abdominal desmoid tumor during the first trimester of pregnancy. Computerized tomography (CT) and transabdominal ultrasound (US) noted a mass within the left rectus sheath measuring up to 15 cm in greatest diameter, with mild compression of the uterus. Preoperative diagnosis was confirmed by core-needle biopsy of the lesion. At 20-weeks gestation, wide local resection of the tumor with disease-free margins, as well as abdominal wall reconstruction with polytetrafluoroethylene (PTFE) mesh was successfully undertaken. Histological examination of the tumor ex vivo confirmed that the lesion was a desmoid tumor consisting of spindle cells with dense infiltrating collagenous fibers. Subsequent to her resection, the patient completed a full-term pregnancy without complication, and proceeded with a complication-free transvaginal delivery at 39 weeks. This case illustrates the probable contribution of estrogens towards desmoid tumor development, the durability of abdominal wall reconstruction when subjected to the extraordinary strain of both a gravid uterus and labor, as well as the safety and efficacy of aggressive surgical therapy during pregnancy.

Forearm Mass in an Adolescent

Forearm Mass in an Adolescent

The Role of Postoperative Irradiation in the Treatment of Locally Recurrent Incompletely Resected Extra-Abdominal Desmoid Tumors

Background: To define the efficacy of postoperative irradiation in patients with recurrent extra-abdominal desmoid tumors in whom surgical intervention has resulted in microscopically or grossly positive surgical margins.Methods: A retrospective analysis was performed on all patients referred to the department of radiation oncology at the Detroit Medical Center with a diagnosis of recurrent extra-abdominal desmoid tumor. This analysis includes all patients seen from 1 January 1990 through 31 December 1999. A total of 11 patients were treated to 13 sites. Ten had microscopically positive margins and three had gross residual disease. Three patients were noted to have multifocal disease at the time of initial representation. Local control, survival, follow-up, and subsequent development of new tumors are measured from the last day of treatment with irradiation.Results: Thirteen sites were treated. Seven patients had received chemotherapy/hormonal therapy prior to surgery and/or irradiation. The most commonly used drug was tamoxifen (n=6). The type of radiation delivered included external beam irradiation alone (n=3), combined external beam irradiation and brachytherapy (n=4), brachytherapy alone (n=3) and 252-Cf neutron brachytherapy alone (n=3). Follow-up has ranged from 29 to 115 months (median=76 months). Three patients have failed locally at 17, 24 and 29 months. One of these was treated for gross residual disease. No patient has died of tumor-related causes. Salvage at the failed sites was possible in twom of three with re-irradiation using external neutrons and/or aggressive surgical intervention and systemic therapy. Complications were most often noted to include decrease range in motion, especially in joint areas, and skin reactions which were normal in presentation. In one site there was development soft tissue necrosis.Conclusion: Based on our experience we recommend postoperative irradiation for all recurrent extra-abdominal desmoid lesions with microscopically or grossly positive surgical margins. Furthermore, patients with recurrent desmoid tumors involving the bony structures of the hand or feet are poor candidates for brachytherapy alone. For patients with extremity lesions, brachytherapy may be a reasonable treatment option provided adequate margins around the tumor bed are covered. The continued recommended use of irradiation in this group of patients is warranted.

Extra-abdominal desmoid around the shoulder joint: one of the differential diagnoses of frozen shoulder

Extra-abdominal desmoid tumor is a type of fibromatosis derived from fascial or musculoaponeurotic structures.3,8 When the tumor occurs around joints, movement is restricted, resulting in pain with motion. We report 3 cases of extra-abdominal desmoid tumor around the shoulder misdiagnosed as frozen shoulder.

The Role of Postoperative Irradiation in the Treatment of Locally Recurrent Incompletely Resected Extra-Abdominal Desmoid Tumors

Background: To define the efficacy of postoperative irradiation in patients with recurrent extra-abdominal desmoid tumors in whom surgical intervention has resulted in microscopically or grossly positive surgical margins. Methods: A retrospective analysis was performed on all patients referred to the department of radiation oncology at the Detroit Medical Center with a diagnosis of recurrent extra-abdominal desmoid tumor. This analysis includes all patients seen from 1 January 1990 through 31 December 1999. A total of 11 patients were treated to 13 sites. Ten had microscopically positive margins and three had gross residual disease. Three patients were noted to have multifocal disease at the time of initial representation. Local control, survival, follow-up, and subsequent development of new tumors are measured from the last day of treatment with irradiation. Results: Thirteen sites were treated. Seven patients had received chemotherapy/hormonal therapy prior to surgery and/or irradiation. The most commonly used drug was tamoxifen (n=6). The type of radiation delivered included external beam irradiation alone (n=3), combined external beam irradiation and brachytherapy (n=4), brachytherapy alone (n=3) and 252-Cf neutron brachytherapy alone (n=3). Follow-up has ranged from 29 to 115 months (median=76 months). Three patients have failed locally at 17, 24 and 29 months. One of these was treated for gross residual disease. No patient has died of tumor-related causes. Salvage at the failed sites was possible in twom of three with re-irradiation using external neutrons and/or aggressive surgical intervention and systemic therapy. Complications were most often noted to include decrease range in motion, especially in joint areas, and skin reactions which were normal in presentation. In one site there was development soft tissue necrosis. Conclusion: Based on our experience we recommend postoperative irradiation for all recurrent extra-abdominal desmoid lesions with microscopically or grossly positive surgical margins. Furthermore, patients with recurrent desmoid tumors involving the bony structures of the hand or feet are poor candidates for brachytherapy alone. For patients with extremity lesions, brachytherapy may be a reasonable treatment option provided adequate margins around the tumor bed are covered. The continued recommended use of irradiation in this group of patients is warranted.

Shoulder function after subtotal excision of the deltoid muscle and of the deltoid and infraspinatus muscles in extra-abdominal desmoid tumor: a long-term follow-up in two cases

Shoulder function after subtotal excision of the deltoid muscle and of the deltoid and infraspinatus muscles in extra-abdominal desmoid tumor: a long-term follow-up in two cases

Multiple operative treatment for extra-abdominal desmoid tumor

Multiple operative treatment for extra-abdominal desmoid tumor

Extraabdominal desmoid tumor in a surgical scar of a patient with sprengel’s deformity

Patients with an extraabdominal desmoid tumor have multiple minor bone abnormalities. The authors describe a rare case of an extraabdominal desmoid tumor that developed in a scar 2 years after surgery for Sprengel’s deformity of the right shoulder in an 8-year-old girl. The association between the tumor and Sprengel’s deformity has never been reported previously. Antecedent surgical trauma might play a role in the development of this tumor.

Quality of surgery and outcome in extra-abdominal aggressive fibromatosis: a series of patients surgically treated at a single institution.

To explore prognostic factors in surgically treated aggressive fibromatosis (extra-abdominal desmoid tumor). A total of 203 consecutive patients treated with surgery over a 35-year period at a single referral center were retrospectively reviewed. One hundred twenty-eight were first seen at our institution with primary disease, whereas 75 had a recurrent tumor. All patients underwent macroscopically complete resection. Margins were rated as negative in 146 (97 with primary tumors, 49 with recurrences) and positive in 57 (31 in primary, 26 in recurrences) patients. Median follow-up was 135 months. Patients with primary disease had a better disease-free survival rate than those with recurrence (76% v 59% at 10 years). Presenting with a recurrence was also the strongest predictor of local failure in the multivariate analysis. In patients first treated for primary disease, size and site had prognostic significance, whereas microscopically positive surgical margins did not. In contrast, in patients with recurrence, there was a trend toward better prognosis if margins were negative (although this was not significant at multivariate analysis). Presence of microscopic disease does not necessarily affect long-term disease-free survival in patients with primary presentation of extra-abdominal desmoid tumors. Thus, function-sparing surgery may be a reasonable choice when feasible without leaving macroscopic residual disease. In patients with recurrences, positive margins may more clearly affect prognosis, potentially necessitating adjuvant radiation in selected cases.

Abdominal wall and foot reconstruction after extensive desmoid tumor resection with free tissue transfer

Desmoid tumors are rare connective tissue tumors, also referred to as deep, aggressive fibromatosis. Although histologically benign, they show an invasive growth behavior and have a high local recurrence rate. The treatment of choice is surgical resection with wide negative margins, while the use of radiotherapy remains controversial. Wide resection of greater desmoid tumors may result in considerable defects and functional impairment. Few papers discuss different options for defect coverage after desmoid tumor resection. Two cases of extensive desmoid tumors, one at the trunk, one at the foot, both with compromised wound margins due to multiple previous surgeries, are presented. To achieve a stable and functional soft tissue cover, the defects were treated with microvascular soft tissue transfer (one free latissimus dorsi, one free radial forearm flap). Both flaps healed uneventfully and patients regained full function of the abdominal wall and foot, respectively. The presented cases demonstrate the efficacy of free flap coverage as an ultimate therapeutic option in selected cases of critical defects after extra-abdominal desmoid tumor resection. Furthermore, free flaps provide a well vascularized ground for adjuvant radiotherapy.

Extra‐abdominal desmoid tumor presenting as an intrathoracic tumor: Case report and literature review

A case of an extra-abdominal desmoid tumor presenting as an intrathoracic tumor (intrathoracic desmoid tumor) in a 46-year-old woman is reported. The tumor originated in the left chest wall and protruded into the left pleural cavity. Simple resection was carried out. The tumor, measuring 13 x 9 x 7 cm, was solid, gray-tan in color, and covered with parietal pleura. Histologically, the tumor was composed of a hypocellular arrangement of spindle-shaped cells with a fibromyxoid background. In some areas, keloid-like hyalinized collagen fibers proliferated, and a perivascular hypercellular area was seen. Immunohistochemical analysis showed that the cytoplasms of the tumor cells were strongly positive for vimentin, and some tumor cells were positive for alpha-smooth muscle actin, but all tumor cells were negative for CD34. These findings were consistent with the characteristics of an intrathoracic desmoid tumor. The differential diagnoses, in particular solitary fibrous tumor and tumors with a myofibroblastic nature, are discussed.

Imaging of musculoskeletal fibromatosis.

The musculoskeletal fibromatoses comprise a wide range of lesions with a common histopathologic appearance. They can be divided into two major groups: superficial and deep. The superficial fibromatoses are typically small, slow-growing lesions and include palmar fibromatosis, plantar fibromatosis, juvenile aponeurotic fibroma, and infantile digital fibroma. The deep fibromatoses are commonly large, may grow rapidly, and are more aggressive. They include infantile myofibromatosis, fibromatosis colli, extraabdominal desmoid tumor, and aggressive infantile fibromatosis. Radiographs typically reveal a nonspecific soft-tissue mass, and calcification is common only in juvenile aponeurotic fibroma. Advanced imaging (ultrasonography, computed tomography, and magnetic resonance [MR] imaging) demonstrates lesion extent. Involvement of adjacent structures is common, reflecting the infiltrative growth pattern often seen in these lesions. MR imaging may show characteristic features of prominent low to intermediate signal intensity and bands of low signal intensity representing highly collagenized tissue. However, fibromatoses with less collagen and more cellularity may have nonspecific high signal intensity on T2-weighted images. Local recurrence is frequent after surgical resection due to the aggressive lesion growth. It is important for radiologists to recognize the imaging characteristics of musculoskeletal fibromatoses to help guide the often difficult and protracted therapy and management of these lesions.

Cytokines associated with the pathophysiology of aggressive fibromatosis.

The rare benign extra-abdominal desmoid tumor is characterized by aggressive invasion of normal tissue. Treatment is complicated by its recurrence, invasiveness, and persistence. The etiology is unknown and the pathophysiology is obscure. Because of exuberant fibroblastic proliferation with collagenous tissue being the primary tissue component, this desmoid tumor has been compared with keloids arising from excessive scar formation in healing wounds. Numerous cytokines are associated with signaling for growth and maintenance of mesenchymal cells. Altered expression of these proteins is associated with many pathologic conditions. It has been proposed that the enhanced expression of platelet-derived growth factor and its receptor characterize desmoid tumors. We tested the hypothesis that the exuberant fibrosis of desmoid tumors may have resulted from the initiation of the cascade of molecular events producing increased expression of cytokines. We used immunohistochemical analysis of cytokines in desmoid tumors compared with keloids and skin to localize the expression of cytokines. The results showed localized increased expression of the cytokines epidermal growth factor, transforming growth factor-beta, tumor necrosis factor-alpha, vascular endothelial growth factor, interleukin-1beta, and interleukin-6 in the endothelial cells of blood vessels in the tumors. Production of tumor necrosis factor-alpha and interleukin-1beta in tumor tissue was increased, but we did not find increased expression of platelet-derived growth factor. We concluded that the increased expression of cytokines associated with angiogenesis usually found in wound healing and invasive tumors may contribute to the pathophysiology of the desmoid tumor.

Extraabdominal Desmoid Tumor

One hundred three patients with extraabdominal desmoid tumor were treated between 1970 and 1996 at the authors' institution. Among these, 15 patients were lost to followup and five were excluded because they had less than 1-year followup. The remaining 83 patients were followed up for a mean of 11.2 years. Thirty-seven (44.6%) patients experienced local recurrence on average 1.8 years after treatment. There was no difference in the incidence of recurrence between the two groups treated with surgery only (45.3%) or with adjuvant radiation therapy administered after inadequate surgical resection of the tumor margins (41.2%). Recurrence was not related to age, gender, and site. None of the 83 patients died of the disease. For recurrent but stable lesions, clinical observation alone may be considered.

耳下部に発生した乳幼児のデスモイド腫よう例

A case of infant extra-abdominal desmoid tumor occurring in the left parotid portion, which has been followed up for 9 years after surgical removal, is reported.A one year and 5 month-old girl visited our clinic on November, 1989, presenting a painless mass in the left parotid portion. Physical examinations showed that the mass was 4×3cm in size, elastic hard in palpation with no facial paresis.Imaging examinations including a CT scan and MRL showed that the mass was located in the parotid portion and extended into the deep Portion of parotid.To confirm the histopathological diagnosis, an open biopsy was performed to locate the desmoid tumor. Then a parotidectomy was performed for surgical removal of the tumor. The tumuor was located in the superior lobe of parotid and could be removed from the facial nerve. The patient has been followed up in our clinic and no reccurence has been observed to date.The desmoid tumor was first described by Macfarlene in 1882, as an abdominal wall tumor. Since that time, reports of this type of tumor arising from places other than the abdominal wall, have been made; these tumors have been called extra-abdominal desmoid tumors. These extra-abdominal desmoid tumors are rarely reported in the face and head region; tumors in the parotid portion of the face are extremely rare.We discussed briefly histopathological findings, age incidence, anatomical location, diagnosis and recurrence of this type of tumor.

上顎歯槽部に発生した腹壁外類けん腫の１例

Extra-abdominal tumors rarely occur in the oral cavity. A case of extraabdominaldesmoid tumor of the maxilla is reported. A 70-year-old woman presented with gingiva swelling of the left upper molar, region. The swelling had been growing rapidly. Radiographs showed resorption of the upper alveolar bone. We suspected a malignant tumor of the maxilla. Partial resection of the left maxilla was performed with the patient under general anesthesia. There has been no evidence of recurrence or metastasis as of about 2 years 2 months after the operation. Histopathologically, the tumor consisted mainly of well differentiated fibroblasts with abundant collagen between the tumor cells. The cells showed no atypia or abnormal mitosis, and oppressively invaded the neighboring osseous tissue. The final diagnosis was extra-abdominal desmoid tumor.

A CASE OF EXTRA-ABDOMINAL DESMOID TUMOR OF THE LATERAL CHEST WALL

A 44-year-old man with was seen at the hospital because of a tumor in the left lateral chest wall. The tumor was located in the 8th rib of left lateal chest to scapla. Intraoperative exsicional biopsy was conduted, which ruled out any malignant neoplasmas. Complete radical tumorectomy was carried out. Histologically, no atypical spindle-shapd fibroblasts with abundant collagen had grown, and the tumor infiltrated into the muscle and scapla on a permanent paraffin block. The definite diagnosis of extraabdominal desmoid tumor was made. There has been no evidence of recurrence, as of 6 months after the surgery. Extra-abdominal desmoid tumors of the chest wall are rare an only 17 cases have been reported in Japan. In a review of the literature, local recurrence occurs so frequently that the complete surgery should be the treatment of this tumor. We presented a case of extra-abdominal desmoid tumor with a review of the literature in Japan. The tumor including the first and second ris which was 8.0×6.0×2.5cm in size was resected en bloc.

Extraabdominal desmoid tumor

An extraabdominal desmoid tumor of the shoulder occurring in a middle-aged woman without Gardner's syndrome is described. Two punch biopsy specimens from the tumor were initially interpreted as representing scar tissue; a third incisional biopsy specimen demonstrated the characteristic features of a desmoid tumor. Because desmoid tumors are locally aggressive, early diagnosis and treatment are crucial to minimize morbidity and mortality. Typical clinical and histologic findings characteristic of an extraabdominal desmoid tumor are described, and treatment options are reviewed.

Two cases of extra-abdominal desmoid tumor in the neck

Two cases of extra-abdominal desmoid tumor in the neck

Desmoplastic fibroma of bone: a report of 18 cases

Desmoplastic fibroma is a rare benign primary bone tumor, histologically identical to the extra-abdominal desmoid tumor of soft tissues. The incidence in major series of bone tumors is 0.1%-0.3%. In the present study 18 cases from the files of the Netherlands Committee on Bone Tumors are reported, with an emphasis on the radiographic features. Eight female and ten male subjects were affected, with a fairly even age distribution from the second to the seventh decade of life. The most frequent sites of involvement were the femur and the pelvis. Radiographically the lesions nearly always demonstrated benign features with respect to pattern of destruction, margins, and reactive bone formation; however, soft tissue extension was sometimes present. Although desmoplastic fibroma usually presents with pain or swelling, in 5 of the 18 cases the existence of the lesion was an accidental finding, and in 2 cases the patient had long-standing, vague complaints of pain. Though overall benign radiographic features may support the final diagnosis, the main and often troublesome histological differential diagnosis is of a low-grade fibrosoma. En bloc resection is the treatment of choice in view of the high incidence of recurrence after curettage.