Cutaneous pleomorphic fibromas arising in patients with germline TP53 mutations.

Pleomorphic fibromas are rare benign cutaneous neoplasms associated with deletion/loss of chromosomes 13q and 17p, where RB1 and TP53 are located, respectively. Herein, we report five cases of pleomorphic fibroma arising in patients with germline TP53 mutations, suggesting a potential link with Li-Fraumeni syndrome. All three patients were female and young (mean age 27) with a strong personal and/or family oncologic history and confirmed pathogenic germline TP53 mutations. In two patients, multiple pleomorphic fibromas were diagnosed. Clinically, the lesions arose at various cutaneous sites and were small (≤2 cm) and raised (4/5). Histopathologically, the tumors were paucicellular, composed of atypical spindled to stellate cells with hyperchromatic and variably pleomorphic nuclei. Mitotic activity was exceedingly low, although rare atypical mitotic figures were seen in one case. Immunohistochemically, the tumor cells were diffusely positive for p16 (3/3) and showed loss of Rb expression (5/5). All cases showed aberrant p53 expression (overexpression in 4, complete loss in 1). The tumors have followed a benign clinical course with no evidence of progression or recurrence. In conclusion, the development of multiple pleomorphic fibromas in a young patient may be a clue to an underlying genetic cancer syndrome involving TP53.

DEGRO guidelines for the radiotherapy of non-malignant disorders : part III: hyperproliferative disorders.

BACKGROUND AND PURPOSE: Radiation therapy (RT) is an established and effective treatment modality in the management of a large variety of hyperproliferative disorders and benign neoplasms. Objective of this article is to summarize the updated DEGRO consensus S2e guideline recommendations. MATERIALS AND METHODS: This report comprises an overview of the relevant aspects of the updated guidelines with regard to treatment decision, dose prescription, and RT technique for a selected group of disorders including Morbus Dupuytren (MD)/Morbus Ledderhose (ML), keloids, Peyronie's disease (induratio penis plastica, IPP), desmoid tumors, pigmented villonodular synovitis (PVNS), symptomatic vertebral hemangiomas (sVH), and Gorham-Stout syndrome (GSS). On the basis of results in the literature, we attempted to classify the level of evidence (LoE) and the grade of recommendation (GR) according to the Oxford criteria. RESULTS: There is comprehensive evidence in the literature that RT is a reasonable and effective treatment modality for the treatment of all the above-mentioned disorders. The LoE varies from 2c to 4, and GR varies from A to C. CONCLUSIONS: The use of RT can be recommended for the interdisciplinary management of most of the reported disorders. It can be used in the primary treatment approach and as an effective adjunct to other treatment modalities or in some indications as a valuable alternative treatment option. We hope that the updated DEGRO S2e consensus guideline recommendations are a helpful tool for radiation oncologists in the clinical decision-making process.

Radiotherapy for Plantar Fibromas (Ledderhose Disease).

Ledderhose disease (plantar fibromas) is histologically related to Dupuytren disease, which has been successfully treated for years with radiotherapy. Many conservative treatments have been advanced for plantar fibromas, including accommodative orthotic devices, which help but do not cure the disease. Surgery is considered the mainstay of treatment for this malady, but the failure rate has been as high as 100%, depending on the type of fasciectomy. Radiotherapy is a new, exciting modality that has shown promising results for treating plantar fibromas.

Radiation therapy for early stages of morbus Ledderhose.

PURPOSE: To evaluate the efficacy of radiation therapy (RT) in the treatment of early stages of benign plantar fibromatosis (Morbus Ledderhose [ML]). PATIENTS AND METHODS: From 2003 to 2008, 24 patients (33 sites) with a mean age of 52 years received RT for symptomatic ML. Prior to RT, 19 patients complained of pain and 15 had walking difficulties. 21 patients (28 sites) were irradiated with orthovoltage X-rays and three (five sites) received electron-beam irradiation. The RT protocol consisted of five weekly fractions of 3.0 Gy (15 Gy), repeated after 6 weeks to a total dose of 30 Gy in 20 patients (28 sites). In four patients (five sites), two single fractions of 4.0 Gy were applied, repeated at intervals of 4 weeks to total doses of 24-32 Gy. Primary study endpoints were the prevention of disease progression and the avoidance of a surgical intervention. Secondary endpoints were pain relief, improvement of gait, and patients' subjective satisfaction measured with a linear analog scale (LAS). RESULTS: After a median follow-up of 22.5 months, none of the patients experienced a progression of number and size of the lesions or the clinical symptoms. In eleven sites (33.3%) complete remission of cords or nodules occurred, in 18 (54.5%) a reduced number or size was noted, and four sites (12.1%) were unchanged. Pain relief was achieved in 13/19 patients (68.4%), and an improvement of gait abnormalities was noted in 11/15 patients (73.3%). The patients' subjective satisfaction measured by means of the LAS revealed a median improvement of 3.5 points in 22/24 patients (91.6%). Skin or soft tissues toxicities RTOG grade > 2 were not noted. CONCLUSION: RT is effective for treatment of the early stages of ML and may obviate the need for a surgical intervention. Long-term follow-up studies including a larger number of patients are required to define the role of RT in the management of this disorder.

Myxofibrosarcoma that Developed Rapidly in the Breast of an Elderly Man and Recurred Early after Surgery: A Case Report.

BACKGROUND: Myxofibrosarcoma is a rare disease occurring subcutaneously in the limbs. We report a case of a rapidly growing myxofibrosarcoma in the breast of an elderly man that recurred early after surgery. CASE PRESENTATION: A 73-year-old man presented with a breast mass. Physical findings showed a large tumor in the right breast, and malignancy was suspected on ultrasonography. Computed tomography (CT) revealed tumor invasion into the pectoralis major and pectoralis minor muscles. Positron emission tomography/CT showed no abnormality in other organs. Needle biopsy results excluded breast cancer but did not provide a definitive diagnosis. However, the tumor grew rapidly before further results were available, so emergency mastectomy was performed. The final pathological diagnosis was high-grade myxofibrosarcoma. Postoperative radiotherapy was started because of remnant tumor. The wound became worsened and swollen, and needle biopsy 10 days after the start of therapy indicated recurrence. Radical resection and thoracoplasty were performed. Postoperative pathological specimens showed no residual tumor. Radical radiation therapy was resumed. The patient has shown no recurrence after an year. CONCLUSIONS: It is important to consult a soft tissue oncologist for tumors in the breast and perform appropriate examination and treatment if soft tissue tumors cannot be ruled out.

Post-radiation sciatic neuropathy: a case report and review of the literature.

BACKGROUND: Post-radiation peripheral neuropathy has been reported in brachial and cervical plexuses and the femoral nerve. CASE PRESENTATION: We describe a patient who developed post-radiation sciatic neuropathy after approximately 3 years and discuss the pathophysiology, clinical course and treatment options available for the deleterious effects of radiation to peripheral nerves. CONCLUSION: This is the first case of post-radiation involvement of the sciatic nerve reported in the literature.

Patient-reported outcomes after electron radiation treatment for early-stage palmar and plantar fibromatosis.

PURPOSE: Palmar and plantar fibromatosis (PPF) is a progressive connective tissue disorder of the hand/foot that often leads to debilitating functional impairment. In Europe, orthovoltage radiation therapy (RT) has been demonstrated to prevent local disease progression for up to 80% of patients with early-stage PPF. There are limited data reporting outcomes for populations outside of Europe or using electron RT. METHODS AND MATERIALS: Between 2008 and 2013, 44 early-stage PPF cases received RT. RT fields involved clinically defined targets encompassing involved areas (skin changes, cords, nodules) with at least 1.5-cm margins. En face electrons (6-12 MeV) and bolus (0.5-1 cm) were selected individually. Outcomes are reported for patients who participated in an institutional review board-approved standardized questionnaire and chart review. RESULTS: Thirty-three patients received 66 treatments (45 hands/15 feet and 6 reirradiations). Most frequent dose schemes were 21 Gy (3 Gy in 7 fractions) and 30 Gy (3 Gy in 10 fractions with 6- to 8-week breaks after 15 Gy). Median time to follow-up survey was 31 months. Disease progression at any location within or outside the RT treatment field occurred in 20 of 33 patients (61%). Fourteen of 60 sites (23%) developed in-field progression, but 4 sites were successfully reirradiated with final local control in 50 of 60 sites (83%). RT improved pretreatment symptoms of pain with strain at 30 of 37 sites (81%) and itch/burn sensations at 17 of 21 sites (81%). There were no reported grade ≥2 late toxicities even with reirradiation. Patient reported overall success with treatment was 31 of 33 patients (94%). CONCLUSION: PPF is a progressive disease. En face electron RT is an effective therapy that stabilizes or improves symptoms in the majority of patients. Reirradiation can be considered as a treatment option for in-field progression. Patients report minimal toxicity and a high rate of satisfaction with treatment.

Delayed cerebrovascular consequences of radiation to the neck. A clinicopathologic study of a case.

A man had a stroke 27 years after radiation therapy to the neck for treatment of laryngeal papillomas. There were premonitory symptoms suggesting cerebral ischemia. In contrast to many previously reported cases, in our patient there is a strong relationship between radiation therapy and stroke. Pathoanatomic study of the surgical vascular specimen strongly suggests that radiation is a potentially modifiable cause of delayed stroke.

Radiotherapy of aggressive fibromatosis.

The long term results of 24 patients treated with postoperative irradiation for aggressive fibromatosis are presented. Tumour sites were the pelvis (8), chest wall (5), shoulder (5), extremities (4) and head and neck (2). Macroscopic complete resection (R1) was performed in 3 cases. 17 patients presented postoperatively with gross disease (R2), 8 of which were recurrent tumours. 4 patients with inoperable disease had biopsies only. Radiation doses ranged from 28 to 64 Gy at a fractionation of 5 x 2 or 4 x 2.5 Gy/week. 4 patients had external irradiation in combination with 192Ir implants, 2 were irradiated with implants alone. In the combined treatment group, external doses ranged from 28 to 52 Gy and additional interstitial doses from 35 to 50 Gy. 192Ir treatment alone was given with 45 and 57 Gy to the contour of the target volume. The 10 year recurrence free survival rate is 75%. A dose response relationship has been established in the dose range of 30-60 Gy revealing an expected 80% persistent tumour control rate at 60 Gy. A dose volume relationship however, could not be derived from our data. Moderate fibrosis without functional impairment developed in 5 patients (21%). These data support a policy of postoperative radiotherapy with 60 Gy in patients with incompletely excised or gross residual tumour following surgery.

Role of brachytherapy in the management of desmoid tumors.

From 1977 to 1982, fourteen patients with desmoid tumors underwent surgery and brachytherapy. Surgery ranged from biopsy to complete or partial excision of the tumor. Most of these patients had locally advanced tumor or positive margins. A high recurrence rate is expected in such a group if treated by surgery alone. In twelve out of fourteen patients the treatment was considered curative when all disease sites could be encompassed. In the remaining two patients the treatment was considered palliative because the tumor encroaching on to the spinal cord was left untreated. Ten out of twelve curatively treated patients have remained free of recurrence at a minimum of 2 year follow-up. Five of them were followed from 4-6 years. In the palliatively treated group, one patient is alive with active disease at 18 months. Three patients developed complications with wound healing. This experience suggests that surgery and brachytherapy treatment for desmoid tumor results in higher local control than expected from surgery alone in this selected group of patients.

Desmoid tumors: local control and patterns of relapse following radiation therapy.

Desmoid tumors are benign neoplasms, arising from musculoaponeurotic tissues, which tend to be locally infiltrative, resulting in a high rate of local recurrence following surgical resection. Nineteen patients with desmoid tumors underwent radiation therapy at the University of California, San Francisco, between 1970 and 1980. Fifteen patients were referred with local recurrence following one or more surgical resections. Three patients were referred for initial radiation therapy with unresectable tumors, and one patient received planned postoperative irradiation following subtotal tumor resection. At the time of treatment, 8 patients had nonresectable disease measuring greater than 10 cm. Five patients had residual tumor masses measuring 4 to 6 cm, and six had only microscopic disease following resection. The majority of patients were treated to a tumor dose of 50-55 Gy at 1.6 to 1.8 Gy per fraction. With a median follow-up of 8 years, 13 patients remained free of recurrent disease following radiation therapy. The 5 year relapse free survival was 72% with 10 patients continuing to be free of disease 5 to 11 years following therapy. Local control was not related to the amount of disease present at the time of treatment. Of the 6 patients who developed recurrent disease, only 1 patient had a true in-field recurrence. Four patients recurred at the margin of the radiation field 1 to 5 years following therapy. Of these four patients, 3 were successfully salvaged while 1 died as a result of tumor extension into a major vessel. One patient with an extensive mesenteric mass did not respond to therapy and died 1 month post irradiation. The patient with the in-field recurrence and 1 patient with a marginal recurrence were successfully treated with combination chemotherapy. Moderate dose radiation therapy to desmoid tumors can result in lasting local control when surgical resection is not possible. Post operative radiation can improve the rate of local control for patients with a high risk of recurrence. As desmoid tumors tend to be locally infiltrative, fields must be very generous to prevent marginal recurrence. Systemic chemotherapy offers an alternative to ablative surgery in the event of local failure following radiation therapy.

Fibromatoses: from postsurgical surveillance to combined surgery and radiation therapy.

The results of management of two groups of patients with musculoaponeurotic (desmoid tumors) and plantar fibromatoses seen at Massachusetts General Hospital (MGH) during the period 1970-1985 are examined: (a) 26 patients who had had surgical resection for their primary fibromatosis but whose surgical margins were positive and who received no further treatment; and (b) 24 patients who were treated for their primary or recurrent fibromatosis by radiation alone or combined with surgery. For the 26 patients who were only observed, despite the positive surgical margins, 9 have recurred; the actuarial continuous local control rate at 5 years was 68% (a median follow-up of 70 months). Five patients had gross disease left after surgery and all of them failed. Seventeen of 21 patients who had grossly complete resection have local control; the four failures have been salvaged. This result supports the rationale for a no treatment but a thorough and close follow-up policy for patients with positive margins after grossly complete resection of a primary desmoid or fascial fibromatosis. There is no risk of metastasis in these patients and hence the effort toward a conservative policy which defers radiation merits interest and further study. Of the second group, 23 patients were treated for gross disease and one patient for microscopic disease after surgical resection. All of the 10 patients who were treated for primary desmoid tumor have local control. Among the 14 recurrent desmoid tumors there have been five local failures, after treatment by radiation alone or radiation + surgery. Three patients treated by radiation alone are currently scored as incompletely regressed tumors. Accordingly 16 of the 24 patients are scored as local controls without evidence of disease and 19 of the 24 are scored as local control (complete response or partial but stable response).

Radiotherapy and conservative surgery in the management of musculo-aponeurotic fibromatosis.

Fifty-four patients with musculo-aponeurotic fibromatosis treated with surgery, surgery and planned post-operative radiotherapy, or radiotherapy alone between 1936 and 1982 have been retrospectively reviewed. Twenty-seven patients had a previous excision before definitive treatment. All patients in whom surgery was known to be incomplete and who had no further treatment relapsed. Nine patients had a complete surgical excision alone and 1 relapsed. Twenty-nine patients were treated with surgery and post-operative radiotherapy and 7 relapsed. Relapse was associated with small field size, orthovoltage irradiation, and doses less than 50 Gy. Radiotherapy was effective in preventing relapse in 6 of 8 cases incompletely excised and in all of these cases the total dose was more than 50 Gy. In 13 assessable patients with clinically evident disease, 14 fields were treated with radiotherapy. Complete response was achieved in 9 fields (although one subsequently relapsed and 2 had a marginal relapse), partial response in 4, and disease stasis in one. Complete resolution took up to 21 months and total doses ranged from 35.2 Gy to 64 Gy. Radiotherapy is indicated in cases of incomplete excision and inoperable disease. Doses should be radical and fields should be sufficiently generous to encompass the anatomical limits of the infiltrated tissues.

The management of desmoid tumors.

PURPOSE: To determine the efficacy of different treatment modalities for desmoid tumors. MATERIALS AND METHODS: We reviewed the treatment of 40 patients with histologically confirmed desmoid tumors seen at Duke University Medical Center between 1974 and 1990. RESULTS: Radiotherapy was administered to 16 patients (Group I)--14 with recurrent disease s/p surgery and in two as initial treatment. The average size of the irradiated lesions was 9.3 +/- 3.9 X 8.4 +/- 3.5 cm. With a median follow-up of 57.5 months and a median administered dose of 5400 cGy (mean 5286 cGy, range 4960-5620 cGy), local control has been obtained in 15/16 patients (94%). Complete regression (5/16), partial regression (5/16), or stable disease (5/16) was produced in 15 patients while one patient failed and was salvaged via gross total resection. Continued regression has been seen up to 60 months after treatment. Fourteen patients underwent primary gross total resection and two underwent subtotal resection (Group II). None received post-operative radiotherapy. Three of 14 patients (21%) recurred after gross total resection. All three were salvaged with subsequent gross total resection. After subtotal resection, 2/2 patients recurred. With a mean follow-up of 52 months, 14 patients are without evidence of disease, one is dead with disease (unrelated cause of death), and one was lost to follow-up after recurrence. Eight patients have been treated with combinations of chemotherapy, NSAIDS, anti-estrogens, and immunotherapy with mixed results (Group III). A subset of seven patients with retroperitoneal disease taken from all three groups had large tumor burden (mean size 17 X 15 cm), an infiltrative nature, as well as a difficult location. The disease was surgically resectable in three patients. One is without evidence of disease 9 years after gross total resection alone. Disease has been stabilized with radiotherapy in the other two patients after multiple unsuccessful surgical resections. Of four patients with unresectable disease, two are dead of disease, one died of unrelated causes with disease, and regression of disease was obtained in the other with Gamma-interferon after unsuccessful treatment with tamoxifen and vincristine, doxorubicin, and cyclophosphamide chemotherapy. CONCLUSION: Gross total resection is the indicated initial therapy, if it can be performed without significant disfigurement. Radiotherapy is also excellent for obtaining local control, even in patients with a large burden of recurrent disease. Doses in the range of 50 to 55 Gy give a chance of local control equal to that obtained with higher doses previously reported.

The results of radiotherapy for orbital pseudotumor.

Between January 1982 and March 1987, 23 patients (26 orbits) were treated for orbital pseudotumor with radiation therapy at the Department of Radiation Oncology, Hospital of the University of Pennsylvania. The patients were referred for clinical relapse after steroid taper in 70%, no response to steroids in 17%, and no steroid treatment (refused or contraindicated) in 13%. Presenting symptoms/signs included soft tissue swelling in 92% of orbits, pain in 92%, proptosis in 85%, and extraocular muscle dysfunction or ptosis in 69%. Decreased visual acuity was seen in only 19% of orbits. Biopsy was performed in nine patients. Treatment consisted of 2000 cGy in 2 weeks in 10 fractions for all patients. Median follow-up was 41 months, with a mean of 53 months, and a range of 21-92 months. Complete response was documented in 87% of orbits with soft tissue swelling, 82% with proptosis, 78% with extraocular muscle dysfunction, and 75% with pain. Of the five patients with visual acuity defects, three experienced complete recovery. There was no difference in complete response in patients biopsied versus those not biopsied. Overall, 17 orbits have remained in complete orbital response with no further steroid requirement (66%). Three orbits suffered local relapse at some point following radiation therapy and were retreated with steroids. These three orbits had durable local control off steroids at last follow-up (11%). Therefore, 77% of orbits attained durable local control and were steroid independent with radiation therapy alone or radiation therapy followed by steroids for relapse. Only one patient developed systemic lymphoma with follow-up. No pretreatment clinical factor reached statistical significance with respect to prognosis following radiation therapy at the less than or equal to .05 level. There were no significant acute or chronic side effects secondary to treatment. Steroids should continue to be first line treatment for orbital pseudotumor, but radiation therapy has a well-defined role in cases of steroid failure or in patients unable to tolerate steroid therapy.

Desmoid tumors: a 20-year radiotherapy experience.

From 1964 through 1984, 45 patients were referred for radiation therapy for desmoid tumor. Fourteen patients had inoperable lesions, or gross residual disease after incomplete resection. Thirty-one patients received postoperative XRT for positive margins or concern about the adequacy of the margin. The minimum follow-up was 2 years, maximum 22 years, median 7.6 years. No patient was lost to follow-up. The primary site was head and neck in 5, upper extremity in 10, chest wall and back in 8, abdomen 2, pelvis 4, and lower extremity 16. All patients were treated with megavoltage radiation therapy using shrinking field techniques. Large fields received a median dose of 50 Gy in 25 fractions. Boost fields were used in the majority of patients to deliver an additional dose of 7 to 27 Gy. The range of total doses was 50 to 76.2 Gy. Three patients received a boost with neutrons. Analysis of patients with inoperable or gross residual showed tumor control in 10 of 14 with a median follow-up of 9.4 years. Resolution of gross disease occurred at a range of 1/2 to 64.3 months with a median of 9 months. There was no evidence of a higher probability of ultimate control at higher doses. Tumor control was equal for men and women. The ten patients with local control had doses from 50 to 76.2 Gy whereas the four patients with in field failures had tumor doses of 57 to 66.4 Gy. There was no difference in median dose for patients with local control (60.3 Gy) versus those with tumor recurrence (60 Gy). For subclinical disease, 31 patients receiving postoperative or preoperative XRT had a 77 percent probability of local control in spite of the history of multiple tumor recurrences; local control was achieved in 8 of 9 with negative or uncertain margins and 16 of 22 with positive margins. An analysis of local control as a function of the number of operations revealed that patients referred for adjuvant radiotherapy with no more than two operative procedures had an 88 percent probability of local control, versus 66 percent for more than two operative procedures. All grade 3 complications (defined as requiring surgical intervention or prolonged hospitalization) occurred with doses above 60 Gy. Management of recurrences was successful in 8 of the 11 patients and no patient has died of tumor.(ABSTRACT TRUNCATED AT 400 WORDS)

The TCD50 and regrowth delay assay in human tumor xenografts: differences and implications.

The response to irradiation of five human xenograft cell lines--a malignant paraganglioma, a neurogenic sarcoma, a malignant histiocytoma, a primary lymphoma of the brain, and a squamous cell carcinoma--were tested in nude mice. All mice underwent 5 Gy whole body irradiation prior to xenotransplantation to minimize the residual immune response. The subcutaneous tumors were irradiated at a tumor volume of 120mm3 under acutely hypoxic conditions with single doses between 8 Gy and 80 Gy depending on the expected radiation sensitivity of the tumor line. Endpoints of the study were the tumor control dose 50% (TCD50) and the regrowth delay endpoints growth delay, specific growth delay, and the tumor bed effect corrected specific growth delay. Specific growth delay and corrected specific growth delay at 76% of the TCD50 was used in order to compare the data to previously published data from spheroids. The lowest TCD50 was found in the lymphoma with 24.9 Gy, whereas the TCD50 of the soft tissue sarcomas and the squamous cell carcinoma ranged from 57.8 Gy to 65.6 Gy. The isoeffective dose levels for the induction of 30 days growth delay, a specific growth delay of 3, and a corrected specific growth delay of 3 ranged from 15.5 Gy (ECL1) to 37.1 Gy (FADU), from 7.2 Gy (ENE2) to 45.6 Gy (EPG1) and from 9.2 Gy (ENE2) to 37.6 Gy (EPG1), respectively. The corrected specific growth delay at 76% of the TCD50 was correlated with the number of tumor rescue units per 100 cells in spheroids, which was available for three tumor lines, and with the tumor doubling time in xenografts (n = 5). The TCD50 values corresponded better to the clinical experience than the regrowth delay data. There was no correlation between TCD50 and any of the regrowth delay endpoints. This missing correlation was most likely a result of large differences in the number of tumor rescue units in human xenografts of the same size.

Nonsurgical management of children with recurrent or unresectable fibromatosis.

At The Children's Hospital of Philadelphia, since 1971, six children 3 months to 17 years of age with fibromatosis have been treated with a combination of vincristine, actinomycin D, and cyclophosphamide (VAC). The first three patients also received radiation therapy (5,500 rads). Locally recurrent tumors developed in four of the children after previous operative removal; the other two had tumors that could not be removed initially. The tumors arose in the neck (three patients), pelvis (two patients), or foot (one patient). In the three patients treated with VAC alone, complete disappearance of tumor was confirmed at second operation in two, and greater than 75% shrinkage on CT scans occurred in the third, all at 4 to 6 months after VAC was started. In two of the three patients who received VAC plus radiation therapy, complete disappearance of tumor occurred at 13 and 16 months; the third had no response. Five of the six patients are free of recurrent fibromatosis at 1, 2, 4, and 11 years after VAC was begun; the sixth has required multiple operations during the last 6 years. We conclude that combination chemotherapy with VAC can produce regression of fibromatosis in some children with recurrent or unresectable lesions. The administration of VAC should be considered for children with fibromatosis in whom operative removal is not feasible, would prove mutilating, or is unlikely to produce long-term control of the disease.

The role of radiotherapy in the treatment of desmoid tumours.

From 1974 to 1983 in the Netherlands Cancer Institute, 21 patients with desmoid tumours were treated with radiation therapy. Nineteen patients were irradiated postoperatively (11 patients had micro- or macroscopic residual disease, 8 patients treated for recurrent disease had narrow surgical margins), 2 patients with inoperable tumours were treated with radiation alone. The entire involved muscle received a dose of 40 Gy, while a boost of 20 Gy was delivered to the tumour bed. Local control was achieved in 19 out of 21 patients, with an actuarial 5 year disease-free survival of 90%. No relation could be found between the amount of tumour present and local control. With careful set-up of treatment fields and long-term physical therapy, complications like fibrosis, ankylosis and oedema could be minimised. These excellent results with radiotherapy for minimal residual tumour, or even for macroscopic tumour, makes mutilating surgery unnecessary.