Inoperable Desmoid Tumors Research Articles

Thoracic Erector Spinae Block as Treatment for Pain Related to Inoperable Desmoid Fibromatosis: A Case Report

BACKGROUND: Desmoid fibromatosis is a rare, sarcoma-like neoplasm that aggressively invades surrounding soft tissue and is often quite painful. Surgery has historically been the mainstay of treatment. Given the high rates of recurrence and unpredictable nature of the lesions, multidisciplinary therapy may be needed to provide adequate analgesia. CASE REPORT: A 35-year-old woman with a history of inoperable desmoid fibromatosis was evaluated in our chronic pain clinic for chest wall and axillary pain due to tumor recurrence. An erector spinae plane block markedly improved her level of pain and functional status. CONCLUSION: This case report highlights the use of a thoracic erector spinae plane block (ESPB) for an inoperable desmoid tumor of the chest wall. Thoracic ESPB is growing in popularity in the field of interventional pain management, but has few definitive indications due to its relative newness. This case report demonstrates that a thoracic ESPB can be a viable, multiregional pain relief strategy for chest wall tumors since our patient reported enduring pain relief, an improved quality of life, and decreased opioid use as a result of the procedure. KEY WORDS: Erector spinae plane block, desmoid fibromatosis, chronic pain, desmoid tumor, regional anesthesia, case report

Efficacy of vinorelbine combined with low-dose methotrexate for treatment of inoperable desmoid tumor and prognostic factor analysis.

To assess the efficacy of conservative chemotherapy for inoperable desmoid tumor (DT) and analyze the prognostic factors. From November 2008 to April 2016, 71 patients of inoperable DT were treated with vinorelbine and low-dose methotrexate in the Department of Bone and Soft Tissue Tumors, Peking University Cancer Hospital & Institute, and enrolled in this retrospective study. The chemotherapy duration is one year. The efficacy of chemotherapy and the prognosis were observed. Of the 71 patients, 55% were female. Age of onset varied from 1 to 47 years, and the median age was 14 years. Only 11 (15.5%) cases suffered primary tumor. The distribution of the site of tumors was: 31 (43.7%) in the trunk, 36 (50.7%) in the limbs, and 4 (5.6%) in the peritoneal and pelvic cavity. The size of tumor (the maximum diameter) differed from 2 to 37 cm with a mean of 9.3 cm. The median follow-up duration was 28 (range, 6-87) months. Common side effects included: nausea and vomiting, liver injury, bone marrow suppression and oral ulcers. When the chemotherapy finished, 1 (1.4%) case achieved complete response, 24 (33.8%) achieved partial response, 37 (52.1%) achieved stable disease and 9 (12.7%) had progressive disease. The overall response rate was 87.3%. The progression-free survival (PFS) of the participants were from 6 to 87 months, and the 2-, 3- and 5-year PFS was 79.9%, 68.4% and 36.3%, respectively. No significant difference was identified in PFS in subgroups of gender, age of onset, age of chemotherapy, tumor site and tumor size. For recurrent, inoperable and progressive DT, enough course of chemotherapy with vinorelbine combined with low-dose methotrexate was an optional choice for local control.

Desmoid Tumors of the Pelvis and Abdominal Wall

In Brief Background: Desmoid tumors are rare, benign, locally invasive neoplasms characterized by proliferation of fibroblasts without cytologic features of malignancy. Wide excision is the recommended primary therapy. Adjunctive radiation therapy may be helpful in patients with positive surgical margins or recurrence. Progestins and tamoxifen are noted to be beneficial in recurrent or inoperable desmoid tumors. Case Reports: A 20-year-old woman with desmoid tumors of the rectus muscles associated with pregnancy is treated by surgical excision alone. A 32-year-old woman with a paravaginal desmoid involving the levator ani muscle was successfully managed with a combination of surgical excision and adjunctive radiation therapy. Discussion: The optimal primary therapy for desmoid tumors is wide, radical, local excision. Other treatments have included radiation therapy, tamoxifen, medroxyprogesterone acetate, corticosteroids, antibiotics, phenylbutazone, indomethacin, ascorbate, and cytotoxic chemotherapy agents. Desmoid tumors have been associated with Familial Adenomatous Polyposis and Gardner's Syndrome, suggesting a genetic predisposition to the disease. The occurrence of these tumors has also been associated with pregnancy and soft tissue trauma. Conclusion: Although historically the primary management of aggressive fibromatosis has been by surgical resection, the diffuse nature of this tumor with its overall benign-appearing histologic features often compromises the surgeon's ability to determine and achieve microscopically free, adequately wide surgical margins of resection. We review the role of adjuvant treatments. Although desmoid tumors are rare neoplasms, pelvic surgeons should be familiar with their diagnosis and treatment.

Sequential treatment of recurrent mesenteric desmoid tumor

The optimal management of inoperable desmoid tumors is still unclear. We report a 26 year-old female patient with familial adenomatous polyposis suffering from a recurrent inoperable intraabdominal desmoid tumor and its sequential treatment. Treatment strategies included low-dose tamoxifen (30 mg orally per day), high-dose tamoxifen (90 mg orally per day), and a subsequent combination of goserelin acetate (3.6 mg subcutaneously once every four weeks) plus low-dose tamoxifen, medroxyprogesterone acetate (1000 mg orally per day) and interferon gamma (3 Mio IU subcutaneously 3 times a week). The combination of goserelin acetate and low-dose tamoxifen resulted in a decrease in tumour size and a complete relief of symptoms for 17 months. Thereafter the tumor progressed and again growth was stopped with interferon gamma therapy for another 6 months. All other treatment modalities had no effect. This study demonstrates long-term regression of a desmoid tumor with combined endocrine therapy using goserelin acetate plus tamoxifen. Tumor progression after 17 months was again stopped by a combination of interferon-gamma and goserelin acetate.

Sequential treatment of recurrent mesenteric desmoid tumor

BACKGROUND The optimal management of inoperable desmoid tumors is still unclear. We report a 26 year-old female patient with familial adenomatous polyposis suffering from a recurrent inoperable intraabdominal desmoid tumor and its sequential treatment. METHODS Treatment strategies included low-dose tamoxifen (30 mg orally per day), high-dose tamoxifen (90 mg orally per day), and a subsequent combination of goserelin acetate (3.6 mg subcutaneously once every four weeks) plus low-dose tamoxifen, medroxyprogesterone acetate (1000 mg orally per day) and interferon gamma (3 Mio IU subcutaneously 3 times a week). RESULTS The combination of goserelin acetate and low-dose tamoxifen resulted in a decrease in tumor size and a complete relief of symptoms for 17 months. Thereafter the tumor progressed and again growth was stopped with interferon gamma therapy for another 6 months. All other treatment modalities had no effect. CONCLUSIONS This study demonstrates long-term regression of a desmoid tumor with combined endocrine therapy using goserelin acetate plus tamoxifen. Tumor progression after 17 months was again stopped by a combination of interferon-gamma and goserelin acetate. Cancer 1996;77:1061-5.

Endocrine therapy for desmoid tumors

Two female patients with desmoid tumors (aggressive fibromatosis) showed tumor regression after endocrine therapy. In one patient, tumor response to tamoxifen has been maintained over several years of treatment. In the second patient, who had inoperable mesenteric fibromatosis, the tumor progressed on tamoxifen but regressed after treatment with Zoladex (goserelin acetate, ICI, Melbourne, Australia) and medroxyprogesterone acetate (MPA). To the authors' knowledge this is the first report of the use of Zoladex in the treatment of desmoid tumors. This review of the literature reveals that the biology of this disease is related to the endogenous hormonal environment and that estrogen receptors have been documented in desmoid tumors. Thirty-five cases are identified where endocrine agents have been employed, with a response rate of 51%. Furthermore, tumors may respond to second-line hormonal therapy after failing to respond to initial endocrine treatment. Endocrine treatments have also been used in other disorders of fibroblastic origin. The authors recommend that endocrine treatment be employed in inoperable desmoid tumors or where there has been postsurgical recurrence. In addition, the role for endocrine therapy in other soft tissue neoplasms should be determined.

Testolactone, sulindac, warfarin, and vitamin k 1 for unresectable desmoid tumors

Ten patients with large inoperable desmoid tumors in various body locations were treated with testolactone. Four tumors (40%) responded with major regressions, i.e., more than 50% reduction in volume. Eight patients received nonsteroid anti-inflammatory drugs (indomethacin, sulindac, or sulindac with warfarin and vitamin k 1 [Mephyton]) for periods of 2 to 91 months. There was one major regression, one partial regression, and three instances of tumor growth arrest over periods up to 8 years. Seven patients were treated with nonsteroid anti-inflammatory drugs concurrent with or after testolactone or tamoxifen. There were five major regressions and one partial regression with extensive central necrosis of an enormous intra-abdominal tumor. The last patient has been treated for only 12 months, with no change in tumor volume. It appears that estrogens function as growth factors for desmoid tumors, and that minimization of these effects inhibits tumor growth in some, but not all, cases. In those instances where antiestrogens were not effective as single agents, the tumors usually responded to subsequent nonsteroid anti-inflammatory drug therapy. Withdrawal of estrogen may be followed by inhibition of transcription of genes that support tumor cell proliferation, and sulindac and indomethacin may augment these effects by inhibiting prostaglandin and cyclic AMP synthesis and the activity of protein kinase C. Warfarin may function as a protonophore to acidify the cytoplasm and prevent the alkalinization that is necessary to initiate DNA synthesis and cell cycle progression, again an impairment of the transcription process.