Extra-abdominal Desmoids Research Articles

Diagnostik und Therapie der aggressiven Fibromatose (extraabdominal desmoid) im Kopf-Hals-Bereich*

Desmoid tumours (extraabdominal desmoids, aggressive fibromatosis) are rare connective tissue tumours, that grow infiltratively and destructively. The analysis of the course of disease in three patients suffering from desmoid tumours in the head and neck region (regio submandibularis, regio parotidea, parapharyngeal space) confirmed the data in literature that painless swelling and dysfunction are the first non-specific signs of the disease. Morphologically reactive fibromatosis and fibrosarcoma should be considered (amongst others) in differential diagnosis. CT and MR scans are useful in determining the extent of the tumours and help to distinguish the tumour from nerves, vessels and bone. Surgery is the therapy of choice in the head and neck region. Primary surgery was performed in our patients. Due to recurrences two of our patients have been operated on several times including major plastic reconstructive surgery. One patient died three and a half years after primary treatment due to sarcomatous degeneration. A large parapharyngeal recurrence was successfully reduced by percutaneous radiation therapy in our second patient. Follow-up of our third patient was NAD 15 months after resection of a large parapharyngeal/mediastinal process including blocking of two cervical vertebrae. Summing up, desmoid tumours are rare connective tissue tumours in the head and neck region with a destructive biological behaviour similar to malignant tumours and a high recurrence rate. Surgery is the treatment of choice. In recurrences or if the tumour is not properly resectable, radiation must be considered, whereas the chemotherapeutical approach is not yet well established.

Desmoid Tumor of the Paraspinous Muscle Involving the Chest Wall

Desmoid tumors of the chest wall are rare lesions with those which involve the paraspinous muscle being particularly rare. This report presents a case of successful resection of a desmoid arising in the right paraspinous muscle. A large ipsilateral latissimus dorsi musculocutaneous flap was used to reconstruct the resulting chest wall defect. Radiation therapy was used postoperatively since the resected margin of the neoplasm was so close to the spine. A review of the literature which discusses possible etiologies of and methods of therapy for extra-abdominal desmoids is presented.

Aggressive fibromatosis (extra-abdominal desmoids) of the head and neck

Five cases of aggressive fibromatosis of the head and neck area (extra-abdominal desmoids) are presented. The clinical and histological character of local aggressiveness of these tumours is emphasised. Radical surgery, when feasible, is the only satisfactory form of treatment. Excision of as much tumour tissue as preservation of vital structures in the area will permit can also offer worthwhile palliation.

Abdominal and extra-abdominal desmoid tumors. Report of 21 cases.

Desmoid tumors are easily diagnosed when located within the muscles of the abdominal wall. On the contrary, extra-abdominal desmoids, because of their various sites and lack of pathognomonic signs, can be very difficult to diagnose. A review of 21 cases of the Istituto Nazionale Tumori of Milan confirms that these tumors primarily affect young, multiparous women and that even extra-abdominal desmoids are prevalent in women. All patients were treated by radical surgery consisting of wide exicision in 18 cases, hemimandibulectomy in 1 case, and amputation of the lower limb in 2 cases. Radical surgery resulted in no recurrences in all cases but one. Our results are in contrast with the relatively high recurrence rates reported in the literature.

The fibromatoses. A histological classification of 156 cases

This study was based on 156 cases of fibromatoses seen at the Soft Tissue Branch of the Armed Forces Institute of Pathology, the Australian Soft Tissue Tumour Registry and the Institute of Medical and Veterinary Science, Adelaide. There were 86 'adult' fibromatoses, 50 'juvenile' fibromatoses, and 20 unclassified lesions. 'Adult' fibromatoses, found predominantly in patients older than 20 yr, fell into 2 main groups; the Dupuytren type fibromatoses (27) and the desmoid tumours (59). In the former group, there were 5 cases of palmar fibromatosis, 15 of plantar fibromatosis, 3 knuckle pads (dorsal fibromatosis), 4 lesions resembling palmar fibromatosis in unusual locations, and no cases of Peyronie's disease. In the desmoid group, there were 13 abdominal desmoids, 39 extra-abdominal desmoids, one mesenteric fibromatosis, 3 examples of multiple extra-abdominal desmoids, one of abdominal desmoid and mesenteric fibromatosis, and 2 of Gardner's syndrome. Six desmoids occurred in scars. There were no cases of multiple familial desmoids. 'Juvenile' fibromatoses occurred predominantly or exclusively in the first 2 decades of life. There were 11 congenital 'fibrosarcomas', 2 congenital generalized fibrornatoses, 8 solitary lesions resembling congenital generalized fibromatoses, 4 fibromatosis colli, 3 diffuse infantile fibromatoses, 5 fibrous hamartomas of infancy, 5 recurring digital tumours of childhood and 12 juvenile aponeurotic fibromas. As a group, the fibromatoses are best regarded as non-metastasizing proliferations of fibrous tissue which may recur locally after excision. They may be both multiple and familial, their growth rate frequently decreases after an initial period of rapid growth, and they may regress spontaneously. This study was based on 156 cases of fibromatoses seen at the Soft Tissue Branch of the Armed Forces Institute of Pathology, the Australian Soft Tissue Tumour Registry and the Institute of Medical and Veterinary Science, Adelaide. There were 86 'adult' fibromatoses, 50 'juvenile' fibromatoses, and 20 unclassified lesions. 'Adult' fibromatoses, found predominantly in patients older than 20 yr, fell into 2 main groups; the Dupuytren type fibromatoses (27) and the desmoid tumours (59). In the former group, there were 5 cases of palmar fibromatosis, 15 of plantar fibromatosis, 3 knuckle pads (dorsal fibromatosis), 4 lesions resembling palmar fibromatosis in unusual locations, and no cases of Peyronie's disease. In the desmoid group, there were 13 abdominal desmoids, 39 extra-abdominal desmoids, one mesenteric fibromatosis, 3 examples of multiple extra-abdominal desmoids, one of abdominal desmoid and mesenteric fibromatosis, and 2 of Gardner's syndrome. Six desmoids occurred in scars. There were no cases of multiple familial desmoids. 'Juvenile' fibromatoses occurred predominantly or exclusively in the first 2 decades of life. There were 11 congenital 'fibrosarcomas', 2 congenital generalized fibrornatoses, 8 solitary lesions resembling congenital generalized fibromatoses, 4 fibromatosis colli, 3 diffuse infantile fibromatoses, 5 fibrous hamartomas of infancy, 5 recurring digital tumours of childhood and 12 juvenile aponeurotic fibromas. As a group, the fibromatoses are best regarded as non-metastasizing proliferations of fibrous tissue which may recur locally after excision. They may be both multiple and familial, their growth rate frequently decreases after an initial period of rapid growth, and they may regress spontaneously.

Soft part sarcomas in negroes.

Fifty-nine patients with soft part sarcomas were seen at Freedmen's Hospital during the years 1949-1969. The sarcomas were classified as: 1. angiosarcoma; 2. extraosseous chondrosarcoma; 3. dermatofibrosarcoma protuberans; 4. fibromatoses (extra-abdominal desmoids); 5. abdominal desmoids; 6. fibrosarcoma; 7. liposarcoma; 8. leiomyosarcoma; 9. myxosarcoma; 10. malignant neurilemmoma; 11. rhabdomyosarcoma, and 12. synovial sarcoma. Tumors of fibrous tissue origin were most common (69%). There were 39 females and 20 males, with an age range from 15 to 81 years. The most common symptom was a painless mass. The lower extremity was involved most often (24 patients). Blood borne metastases were most frequent, but lymph node metastases also occurred. Angiography was of adjunctive value in 2 patients. Biopsy (incisional or excisional) and treatment by wide local excision, muscle group excision, or amputation were mainstays in management. With exception of desmoids and dermatofibrosarcoma protuberans, 5-year survival was quite low. Prompt biopsy of suspicious masses with appropriate therapy offers best chance in these patients. The only observed racial difference was the apparent greater number of fibrous tissue tumors.

Musculo-aponeurotic fibromatosis of the shoulder girdle (extra-abdominal desmoid). Analysis of thirty cases followed up for ten or more years.

Analysis of 30 fibromatoses (extra-abdominal desmoids) arising from the musculo-aponeurotic structures of the shoulder girdle revealed an aggressive clinical course with a tendency to recur in a high percentage of cases. Although all patients were living without evidence of further growth 10 or more years after the discovery of the tumor, local recurrence was observed in 57% of the patients. Recurrence was more common in patients younger than the median age of 23 years and in patients in whom the infiltrating tumor had reached a large size at the time of the first treatment. The microscopic picture, indistinguishable from that of abdominal desmoids, varied little from case to case. Attempts to correlate histology and behavior were unsuccessful. Prompt and complete surgical removal is required to prevent complications from recurrent tumor growth.

Desmoplastic fibroma vs. desmoid tumor of bone. Two cases illustrating a problem in differential diagnosis and classification.

Nichols (10) first called attention to the occurrence of desmoid tumors in extra-abdominal sites (9). With some extra-abdominal desmoids, invasion of contiguous bone has been described (2, 8, 13, 15). In 1958, Jaffe delineated “desmo-plastic fibroma” as a rare primary bone tumor usually found in young adults (7). He did not establish a clear histological distinction between the entity he had thus defined and the soft-tissue extra-abdominal desmoid. There have been only 13 cases (Table I) added to the literature since Jaffe's report (3–6, 11–16). Each of the two tumors presented in this communication shows a soft-tissue mass with an accompanying bony lesion. The first would be considered a desmoplastic fibroma belonging with the group in Table I by the pathologic criteria of some authorities (4, 16), but a periosteal desmoid by others (12). The second represents a soft-tissue desmoid invading bone secondarily. In addition to their radiodiag-nostic interest, these cases will illustrate the problems presente...

Principles in the management of extra-abdominal desmoids.

CancerVolume 13, Issue 4 p. 825-836 ArticleFree Access Principles in the management of extra-abdominal desmoids R. Thomas N. Hunt M.D., R. Thomas N. Hunt M.D. Washington University School of Medicine, Barnes Hospital, and Barnard Free Skin and Cancer Hospital, St. Louis, Mo. The Women's Advertising Club of St. Louis Fellow, Division of Surgical Pathology, Washington University School of Medicine, St. Louis, Mo.Search for more papers by this authorHarry C. Morgan M.D., Harry C. Morgan M.D. Washington University School of Medicine, Barnes Hospital, and Barnard Free Skin and Cancer Hospital, St. Louis, Mo.Search for more papers by this authorLauren V. Ackerman M.D., Lauren V. Ackerman M.D. Washington University School of Medicine, Barnes Hospital, and Barnard Free Skin and Cancer Hospital, St. Louis, Mo.Search for more papers by this author R. Thomas N. Hunt M.D., R. Thomas N. Hunt M.D. Washington University School of Medicine, Barnes Hospital, and Barnard Free Skin and Cancer Hospital, St. Louis, Mo. The Women's Advertising Club of St. Louis Fellow, Division of Surgical Pathology, Washington University School of Medicine, St. Louis, Mo.Search for more papers by this authorHarry C. Morgan M.D., Harry C. Morgan M.D. Washington University School of Medicine, Barnes Hospital, and Barnard Free Skin and Cancer Hospital, St. Louis, Mo.Search for more papers by this authorLauren V. Ackerman M.D., Lauren V. Ackerman M.D. Washington University School of Medicine, Barnes Hospital, and Barnard Free Skin and Cancer Hospital, St. Louis, Mo.Search for more papers by this author First published: July/August 1960 https://doi.org/10.1002/1097-0142(196007/08)13:4<825::AID-CNCR2820130427>3.0.CO;2-ECitations: 94 AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat Citing Literature Volume13, Issue4July/August 1960Pages 825-836 ReferencesRelatedInformation