Special Issue "Desmoid Tumors"

Quicklinks

A special issue of Cancers (ISSN 2072-6694).

Deadline for manuscript submissions: closed (30 October 2011)

Special Issue Editor

Guest Editor
Prof. Dr. Henry J. Mankin

1 Orthopaedic Service, 1122A Jackson Building, Massachusetts General Hospital, Boston, MA 02114, USA
2
Harvard Medical School, 25 Shattuck Street, Boston, MA 02115, USA
Fax: +1 617 724 7396
Interests: cartilage and arthritic disorders; bone allografting; connective tissue tumor biology and treatment; Gaucher disease and other genetic disorders

Special Issue Information

Dear Colleagues,

Desmoid tumors arising in the soft tissue of the extremities are uncommon with probably less than 2 case per million population in the United States. The lesions can be aggressive, can recur or appear in multiple body parts, but do not metastasize.  They are more frequent in young women; and children may have an aggressive form of the disorder.  It is important to note that the lesions may also occur in proximity to the uterus, viscera, colon and even the abdominal wall. Tumors are firm, rubbery to palpation and usually stain with vimentin, alpha actin and desmin.  The cellular elements are classically elongated, slender, spindle shaped cells separated by large numbers of collagen fibers. Desmoid tumors may appear aggressive and in the lower extremities can break through the skin or even into the bone.  Treatment with surgery is the classic approach but recently radiation and even chemotherapy have been found to be very useful for aggressive lesions.

Henry J. Mankin
Guest Editor

Keywords

  • desmoid tumors
  • fibroblastoma
  • myofibroblastoma
  • fibromatosis
  • Gardner's disease

Published Papers (6 papers)

View options order results:
result details:
Displaying articles 1-6
Export citation of selected articles as:

Research

Jump to: Review

Open AccessArticle Surgical Treatment of Intra-Abdominal Desmoid Tumors Resulting In Short Bowel Syndrome
Cancers 2012, 4(1), 31-38; doi:10.3390/cancers4010031
Received: 20 December 2011 / Revised: 11 January 2012 / Accepted: 16 January 2012 / Published: 19 January 2012
Cited by 2 | PDF Full-text (88 KB) | HTML Full-text | XML Full-text
Abstract
Advanced intra-abdominal desmoids tumors present with severe symptoms, complications or rapid growth, which lead to adverse outcomes. Our aim was to evaluate the treatment and outcome of patients with advanced intra-abdominal desmoids tumors, and develop guidelines for surgical management of these patients. We reviewed the clinical courses of 21 adult patients with advanced stage intra-abdominal desmoid tumors who presented to an intestinal rehabilitation and transplantation program. Patients with massive intestinal resection presented in two groups. The first group had a short small intestinal remnant after resection ( < 60 cm). These patients were poor rehabilitation candidates and eventually met criteria for transplant. The second had longer intestinal remnants and were more successfully rehabilitated and have not had complications that would lead to transplantation. Advanced intra-abdominal desmoid tumors have outcomes after resection that merit aggressive resection and planned intestinal rehabilitation and intestinal transplantation as indicated. Full article
(This article belongs to the Special Issue Desmoid Tumors)
Open AccessArticle Treatment of Extra — Abdominal Desmoid Tumors with Chemotherapy
Cancers 2011, 3(3), 3394-3404; doi:10.3390/cancers3033394
Received: 15 August 2011 / Accepted: 18 August 2011 / Published: 25 August 2011
Cited by 2 | PDF Full-text (202 KB) | HTML Full-text | XML Full-text
Abstract
Fibromatosis, or extra-abdominal desmoid tumor, is a benign disease which often has an aggressive clinical course that can be difficult to treat. We performed a retrospective review of 16 patients (12 females and four males) with a mean age of 34.2 years [...] Read more.
Fibromatosis, or extra-abdominal desmoid tumor, is a benign disease which often has an aggressive clinical course that can be difficult to treat. We performed a retrospective review of 16 patients (12 females and four males) with a mean age of 34.2 years treated with methotrexate and vinblastine for newly diagnosed or recurrent extra-abdominal desmoid tumor. The mean age of our patient cohort was 34.2 years (range 11–70), and the mean tumor size was 11.5 cm (range 2.5–21.2 cm). The mean duration of therapy was 12 months with an average follow-up of 43 months (range 1–149 months). Fourteen of 16 patients demonstrated a clinical response to treatment. Eight of 14 patients demonstrated a radiologic decrease in tumor size. Only one patient progressed on therapy. Six patients developed recurrent symptoms after discontinuation of treatment. Chemotherapy-related symptoms including neutropenia, nausea, and vomiting were common and observed in most patients, however these side effects were mild and transient. Five patients developed peripheral neuropathy that prompted a change from vinblastine to vinorelbine during treatment. One potentially life-threatening complication (pneumocystis pneumonia) occurred which was diagnosed early and successfully treated. The use of methotrexate and vinblastine/vinorelbine in the management of fibromatosis appears to be an effective treatment with minimal treatment-related side effects. Full article
(This article belongs to the Special Issue Desmoid Tumors)

Review

Jump to: Research

Open AccessReview Desmoid Tumors in the Pediatric Population
Cancers 2012, 4(1), 295-306; doi:10.3390/cancers4010295
Received: 10 February 2012 / Revised: 29 February 2012 / Accepted: 6 March 2012 / Published: 9 March 2012
Cited by 2 | PDF Full-text (251 KB) | HTML Full-text | XML Full-text
Abstract
Desmoid tumors are benign soft tissue tumors associated with locally aggressive growth and high rates of morbidity, but they do not metastasize via lymphatic or hematogenous routes. While most of the data on desmoid tumors originates in the adult literature, many of [...] Read more.
Desmoid tumors are benign soft tissue tumors associated with locally aggressive growth and high rates of morbidity, but they do not metastasize via lymphatic or hematogenous routes. While most of the data on desmoid tumors originates in the adult literature, many of the findings have been applied to the management of pediatric patients. This article discusses the epidemiology, etiology, clinical presentation, pathology, and treatment of this rare tumor in the pediatric population and includes a literature review of the most recent large series of pediatric patients with desmoid tumors. Full article
(This article belongs to the Special Issue Desmoid Tumors)
Open AccessReview Desmoid Tumors in Pregnant and Postpartum Women
Cancers 2012, 4(1), 184-192; doi:10.3390/cancers4010184
Received: 4 January 2012 / Revised: 11 February 2012 / Accepted: 13 February 2012 / Published: 21 February 2012
Cited by 3 | PDF Full-text (307 KB) | HTML Full-text | XML Full-text
Abstract
We report here a review of the current medical literature on pregnancy associated desmoids, including 10 cases of our own. The pertinent findings are that a large percentage of desmoids in females arise in and around pregnancy. Most occur in the abdominal [...] Read more.
We report here a review of the current medical literature on pregnancy associated desmoids, including 10 cases of our own. The pertinent findings are that a large percentage of desmoids in females arise in and around pregnancy. Most occur in the abdominal muscles, particularly the right rectus abdominus, perhaps related to trauma from abdominal stretching and fetal movement. While these tumors may regress spontaneously after delivery most can be surgically resected with low recurrence rates even with R1 resections and this is clearly the treatment of choice. Subsequent pregnancies do not appear to result in recurrence in either FAP or non FAP patients. It is not clear from currently available data whether pregnancy associated desmoids are molecularly distinct from other desmoids. Full article
(This article belongs to the Special Issue Desmoid Tumors)
Open AccessReview Transition of Treatment for Patients with Extra-Abdominal Desmoid Tumors: Nagoya University Modality
Cancers 2012, 4(1), 88-99; doi:10.3390/cancers4010088
Received: 21 November 2011 / Revised: 4 January 2012 / Accepted: 3 February 2012 / Published: 7 February 2012
Cited by 4 | PDF Full-text (505 KB) | HTML Full-text | XML Full-text
Abstract
Treatment modalities for desmoid tumors have been changed because of the high recurrence rate, even after wide resection, and some cases experience spontaneous self-regression during clinical course. The treatment modality in our institutions before 2003 was surgical resection with wide surgical margin, however, meloxicam, which is a NSAID and a selective COX-2 inhibitor has been applied consecutively since 2003. We reviewed the previously reported outcomes of surgical and conservative treatment in our institutions. Among 30 patients receiving surgical treatment, 16 (53%) recurred. Younger age ( p < 0.05) was a significant poor factor. According to RECIST for meloxicam treatment, CR was in one, PR in 10, SD in eight, PD in one evaluated at 2011. Older age ( p < 0.01) was significantly associated with good outcome for meloxicam treatment. Results of the previous study indicated that surgical treatment alone could not control desmoid tumors, even with negative surgical margin. Considering the functional impairment resulting from surgery with negative surgical margin, a conservative and effective treatment modality with fewer complications is desired. Conservative treatment with meloxicam is a promising novel modality for patients with extra-abdominal desmoid tumors. Full article
(This article belongs to the Special Issue Desmoid Tumors)
Open AccessReview Current Perspectives on Desmoid Tumors: The Mayo Clinic Approach
Cancers 2011, 3(3), 3143-3155; doi:10.3390/cancers3033143
Received: 30 May 2011 / Revised: 6 July 2011 / Accepted: 2 August 2011 / Published: 8 August 2011
Cited by 7 | PDF Full-text (1271 KB) | HTML Full-text | XML Full-text
Abstract
Desmoid tumors are a rare group of locally aggressive, non malignant tumors of fibroblastic origin that can lead to significant morbidity due to local invasion. Despite advances in the understanding of these tumors, their natural history is incompletely understood and the optimal [...] Read more.
Desmoid tumors are a rare group of locally aggressive, non malignant tumors of fibroblastic origin that can lead to significant morbidity due to local invasion. Despite advances in the understanding of these tumors, their natural history is incompletely understood and the optimal treatment is still a matter of debate. Local control is the main goal of treatment and there has been a change in philosophy regarding the management of these tumors from aggressive surgical resection to function preservation. A multidisciplinary approach is essential to plan local control with acceptable morbidity. The current Mayo Clinic algorithm for the treatment of these tumors is based on institutional experience and the available evidence in the literature: asymptomatic/non progressive lesions away from vital structures are managed with observation and regular imaging; primary or recurrent desmoid tumors which are symptomatic or progressive or near vital structures are managed with wide surgical resection when wide surgical margins are possible with minimal functional and cosmetic loss. When positive or close surgical margins are likely, surgical resection with adjuvant radiotherapy or definitive radiotherapy is preferred. If likely functional or cosmetic deficit is unacceptable, radiotherapy is the treatment of choice. Unresectable lesions are considered for radiotherapy, chemotherapy or newer modalities however an unresectable lesion associated with a painful, functionless, infected extremity is managed with an amputation. Full article
(This article belongs to the Special Issue Desmoid Tumors)

Journal Contact

MDPI AG
Cancers Editorial Office
St. Alban-Anlage 66, 4052 Basel, Switzerland
cancers@mdpi.com
Tel. +41 61 683 77 34
Fax: +41 61 302 89 18
Editorial Board
Contact Details Submit to Cancers
Back to Top