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.

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.

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.

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.

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.

Palliation of recurrent myxofibrosarcoma with radiotherapy and hyperthermia.

Soft tissue sarcomas are rare biologically and histologically heterogeneous neoplasms that may arise throughout the body. The preferred treatment is total resection with a sufficient margin. Radiotherapy with or without chemotherapy offers another treatment option, but its effectiveness is limited. Hyperthermia, a treatment method that heats tumour tissue by exposing the target tissues to conductive heat sources or non-ionising radiation, is known to enhance the effect of radiotherapy. We report a case of an elderly man with a second recurrent myxofibrosarcoma in the left groin, who responded well to radiotherapy in combination with hyperthermia. This combination treatment was effective in maintaining the patient's quality of life during his remaining years.

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.

Treatment of aggressive fibromatosis: the experience of a single institution.

AIMS: Aggressive fibromatosis is a locally aggressive infiltrative low-grade tumour, although pathologically benign, and it does not metastasise, yet it can cause serious local distressing symptoms by virtue of local destruction and impairment of local function. The aim of this study was to emphasise the role of radiotherapy and adequate surgery in the treatment of fibromatosis in patients presenting with newly diagnosed or recurrent disease and to analyse our treatment results over 15 years for this rare tumour type. MATERIALS AND METHODS: Fifty-four patients with confirmed diagnosis of aggressive fibromatosis treated at King Faisal Specialist Hospital between 1990 and 2006 were identified from our local cancer registry. Forty-seven patients had surgery: complete resection (R0) in 20 patients, incomplete surgery (R1/2) in 27 patients, and seven patients had biopsy only. Forty-five patients were treated with radiotherapy: 38 patients were treated with postoperative radiotherapy, three patients were treated with preoperative radiotherapy and four patients had radiotherapy as the only treatment. The radiotherapy dose ranged between 45 and 60Gy (median 50.4Gy). Three patients did not receive any form of treatment apart from biopsy, but were still included in the final analysis. RESULTS: Fifty-two per cent (28/54 patients) of our patient population had tumour recurrence when first presented to King Faisal Specialist Hospital. The median age was 29.5 years (range 2-63 years). The most common site of involvement was the extremities (28 patients). Among the 54 patients (with primary and recurrent presentation) there were 10 local recurrences, all of which were within the original primary site. The 5-year progression-free survival and overall survival rates for the whole group were 75 and 95%, respectively. Univariate and multivariate Cox regression analysis showed that the depth of invasion significantly affected progression-free survival. CONCLUSION: Aggressive fibromatosis is effectively treated with surgery and postoperative radiotherapy. Patients first presenting with tumour recurrence may still have local tumour control comparable with newly diagnosed patients.

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.

Recurrence of plantar fibromatosis after plantar fasciectomy: single-center long-term results.

BACKGROUND: Plantar fibromatosis is a rare, hyperproliferative, benign lesion of the plantar aponeurosis with an unknown cause. Surgical treatment is associated with a high recurrence rate and risk of complications. The goal of this study was to determine the recurrence rate of plantar fibromatosis after plantar fasciectomy at the authors' institute during the past three decades and the factors associated with an increased risk for recurrence. METHODS: The study group contained 27 patients with plantar fibromatosis, who underwent 40 operations on 33 feet, including 13 right (39 percent) and 20 left (61 percent) feet. RESULTS: The overall recurrence rate was 60 percent. Treating a primary lesion with total plantar fasciectomy was associated with the lowest (25 percent) and local resection of the lesion was associated with the highest recurrence rate (100 percent). There seemed to be a relation between the existence of multiple nodules in one foot and a higher recurrence rate. The recurrence of a primary lesion treated with fasciectomy combined with postoperative radiotherapy seemed to be lower in comparison with the recurrence rate after surgery only. CONCLUSIONS: Surgical treatment of plantar fibromatosis is associated with a high recurrence rate and indicated only when the lesions are highly symptomatic and conservative measures fail. Total plantar fasciectomy is the most successful treatment in this study, particularly for primary lesions. The role of postoperative radiotherapy should be evaluated further. A prospective multicenter study comparing different surgical procedures will be needed to determine the type of operation that most effectively eliminates plantar fibromatosis.

Incidence and treatment of recurrent plantar fibromatosis by surgery and postoperative radiotherapy.

BACKGROUND: The incidence of plantar fibromatosis (PF) is unknown. Sometimes PF tends to recur repetitively after surgical treatment. In our institute we have used postoperative radiotherapy in an attempt to diminish the change on recurrence. METHODS: The Dutch Network and National Database for Pathology (PALGA) was consulted to establish the incidence of plantar fibromatosis (PF). Data from 9 patients (11 feet) with PF referred to our institute for recurrent disease were analyzed and the role of postoperative radiotherapy in prevention of recurrence was studied. RESULTS: An average of 1.2 operations for PF was performed per 100,000 citizens yearly in the Netherlands. Twenty-six operations were performed and postoperative radiotherapy was used in 6 cases. Plantar fasciectomy was associated with the lowest recurrence rate. After microscopically incomplete excision or excision of early recurrence (< or =6 months) alone all tumors recurred, while recurrence was rarely observed after adjuvant radiotherapy. However, radiotherapy was associated with significantly impaired functional outcome in 3 cases. CONCLUSIONS: Plantar fibromatosis is relatively rare. Plantar fasciectomy seems to be the operation of choice. Although effective in decreasing the recurrence rate, adjuvant radiotherapy should be used very selectively because of its serious side effects.

[Radiation therapy for Morbus Ledderhose -- indication and clinical results]. / Strahlentherapie beim Morbus Ledderhose-Indikation und klinische Ergebnisse.

BACKGROUND: Morbus Ledderhose (ML) is a rare hyperproliferative disorder of the plantar aponeurosis which is similar in its clinical course to Morbus Dupuytren (MD). We examined whether radiotherapy (RT) can effect symptoms and prevent disease progression. PATIENTS AND METHODS: From June 1996 to December 2001, 25 patients (12 female/13 male) aged 9-76 (median: 56) years had radiotherapy (RT) for symptomatic ML. Follow-up (FU) was at least 1 year. 36 feet (16 right/20 left) were treated, as eleven patients had bilateral disease. Twelve (48%) patients had MD. There were 63 nodules (with 0,5-6,5 cm diameter) on all feet and 20 cords (with 1-4 cm length) on 13 (52%) feet prior to RT. 21 (84%) patients had one or more signs: 14 (56%) severe local pain, eight (32%) walking difficulties, twelve (48%) other symptoms, pressure or tension sensation. The RT field involved all nodules and cords plus safety margin. Two RT-series were applied (each 5 3 Gy in 1 week) separated by 8-12 weeks up to a total dose of 30 Gy. Evaluation was performed at the end of RT, after 3 and 12 months FU and in December 2002. The primary endpoint was prevention of disease progression and avoidance of surgery. Secondary endpoints were objective changes of morphological and functional parameters and patient's satisfaction measured on a visual analogue scale (VAS). RESULTS: With a median FU of 38 (12-67) months no patient experienced progression or underwent surgery: 11 of 36 (44%) feet had a reduced number (overall: -16) or size of nodules, 7 of 13 (54%) feet had a reduced number (overall: -9) or length of cords; gait was improved in six of twelve (50%) feet; pain was reduced or had completely disappeared in 9 of 15 (60%) feet, and other symptoms disappeared in 8 of 18 (44%) symptomatic feet. 20 (80%) patients regarded 28 of 36 (78%) treated feet as improved and 8 (22%) in stable condition. The median relative improvement stated by patients on the VAS was 50% (0-100%). Treatment side effects were minimal: During and within 3 months of the RT course only a slight erythema (CTC 1 degrees ) was seen in five treated lesions, while dry skin changes within the RT portal were observed in three cases (11%) in long term FU (> 12 months). CONCLUSIONS: Radiotherapy is effective in treating ML and may prevent otherwise necessary surgical interventions. Nodules, cords and symptoms regress, but long-term outcome of at least 5 years has to be awaited. Prospective phase III studies should confirm these results.

Preoperative radiotherapy is effective in the treatment of fibromatosis.

The use of preoperative radiation is well-established for soft tissue sarcoma, but its use in fibromatosis is not well-characterized. The purpose of this study was to examine the impact of preoperative radiotherapy on the local control of fibromatosis and to assess treatment-related morbidity in this setting. In particular we assessed complication rates in comparison with soft tissue sarcoma treatment. All patients with fibromatosis referred to this unit who received preoperative radiotherapy (50 Gy in 25 fractions) from 1988 to 2000 and who had at least 2 years of followup were included in this study. The rate of recurrence in this group was ascertained. Similarly constructed datasets from all patients with soft tissue sarcomas of the extremities who received preoperative radiation from 1986 to 1997 also were analyzed. The rates of complications in the two groups were compared. Fifty-eight patients were treated with preoperative radiation for fibromatosis and the median followup was 69 months. There were 11 local recurrences (19%). Major wound complications manifested in two patients (3.4%). Wound-related complications arose in 89 of 265 patients with soft tissue sarcomas (33.5%). There was a significant difference in the rate of major wound complications observed in the two groups. The use of radiotherapy before surgery is effective in the combined treatment of fibromatosis.

Radiation treatment for aggressive fibromatosis: findings from observed patterns of local failure.

PURPOSE: We retrospectively evaluated the outcome of patients treated with radiotherapy, with or without surgery, for aggressive fibromatosis. The patterns of local failure were analyzed to determine the optimum radiation dose and volume for irradiation. PATIENTS AND METHODS: Twenty-four patients with histologically confirmed aggressive fibromatosis were treated with radiation therapy at the Yonsei Cancer Center between 1990 and 1998. The radiation dose per patient ranged from 39.6 to 59.4 Gy (mean: 49.4 Gy). The entire operative bed, with a generous margin, was included in the radiotherapy volume. Patients were considered as locally controlled if there was no evidence of the disease during their follow-up period, and if the tumors were stable for more than 2 years. The minimum duration of the follow-up period was 26 months, with a median of 69 months. RESULTS: The actuarial 10-year recurrence-free and overall survival were 88.5 and 100%, respectively. Patients who had a recurrence were salvaged by combined surgery and re-irradiation. Recurrences developed only in patients who had a recurrent disease after surgery and were treated with an inadequate radiation volume. In 9 patients with a gross measurable disease, there were no in-field failures; these had been treated with a median of 50.4 Gy (range: 40-60 Gy) of radiation. Eight patients with a microscopic residual disease were also locally controlled with 41.4-59.4 Gy (median: 45 Gy) of radiation. No patient has developed either secondary malignancy or any serious radiation complications. CONCLUSION: Radiotherapy for aggressive fibromatosis can be an effective treatment option for maintaining a disease-free status. As fibromatosis, with either a microscopic, or a gross residual disease, can be controlled with a moderate dose of radiation, adjuvant postoperative radiotherapy following surgical excision is recommended with the least sufficient margin to preserve good function and cosmesis. The geographic relationship may require a more precise definition; in addition, regardless of the existence of neighborhood normal tissue barriers, a wide coverage of the radiation volume may be needed.

Fibromatosis of the head and neck: a challenging lesion.

Fibromatosis is a histologically benign fibrous neoplasm that arises from the musculoaponeurotic tissues of the body. It can have significant morbidity when it occurs in the head and neck region because of the proximity of vital structures. This review article considers the etiology, demographics, pathology, natural history, and various treatment modalities for this lesion. Therapeutic guidelines are provided.