Perianal Paget disease treated definitively with radiotherapy.

Extramammary Paget disease (empd) is a relatively rare cutaneous disorder described as an apocrine gland tumour occurring in both a benign and a malignant form with metastatic potential. The areas of the body affected are the vulva, perianal region, penis, scrotum, perineum, and axilla, all of which contain apocrine glands. When empd affects the perianal region, it is called perianal Paget disease (ppd). All forms of empd, including ppd, are typically treated by wide surgical excision. Perianal Paget disease usually occurs later in life in patients who are often poor surgical candidates, but the available literature is scarce regarding other treatment modalities, including definitive radiotherapy. We contend that ppd can be safely and effectively treated with radiotherapy, and here, we present the case of a 75-year-old woman with ppd who was successfully so treated. A brief review of the literature concerning the diagnosis, natural history, and treatment of ppd is also included.

Fractionated stereotactic radiotherapy boost and weekly paclitaxel in malignant gliomas clinical and pharmacokinetics results.

Management of Malignant Gliomas continues to be a challenge. We prospectively studied the role of adding weekly Paclitaxel to Fractionated Stereotactic Radiation Therapy (FSRT) in the treatment of Malignant Gliomas. Twenty-three Glioblastoma Multiforme and two Anaplastic Astrocytoma were studied. Patients received 46 Gy at 2 Gy/fraction followed by a boost utilizing FSRT at a fraction of 2.5 Gy for 8 fractions. Paclitaxel is delivered concomitantly at 150 mg/m(2) weekly for six cycles. Eighteen patients had pharmacokinetic assays of Paclitaxel levels. All patients were followed until death or for a maximum of 36 months. The overall survival of the whole group was 14 months. The median survival for RPA prognostic classes III, IV, V, and VI were 20, 14, 12, and 11 months. Higher survival (14 months) was noted in the subtherapeutic phenytoin level group compared to 10 months in the therapeutic group (P=0.271). No grade 4 CTCAE (version 3.0) toxicities were observed. Enhanced survival was demonstrated with gross tumor resection (20.8 months), KPS > or =80 (18.7 months) and age < or =60 years (27 months) as compared to subtotal resection or biopsy (12.1 months, P< 0.005), KPS < or =70 (10.8 months, P=0. 005) and older age > 60 (10.46 months, P=0.006), respectively. Our study suggests that: i) the use of weekly Paclitaxel and FSRT in Gliomas is well tolerated with a survival of 14 months; ii) the regimen resulted in improvement of survival of RPA classes IV, V, VI; and iii) the use of FSRT boost may be studied with other chemotherapeutic agents to see if superior results can be attained.

Commissioning and clinical results utilizing the Gildenberg-Laitinen Adapter Device for X-ray in fractionated stereotactic radiotherapy.

BACKGROUND: The Gildenberg-Laitinen Adapter Device for X-Ray (GLAD-X/LS) frame is a positioning device that allows the use of the same fiducial points as the Brown-Robert-Wells (BRW) system. Thus it permits treatment planning to be accomplished by the Radionics X-knife Radiosurgery Program. We investigated the commissioning and clinical benefits of the GLAD-X/LS for fractionated stereotactic radiotherapy (FSRT) in patients who were unable to tolerate the Gill-Thomas-Cosman (GTC) frame. METHODS AND MATERIALS: Commissioning of the GLAD-X/LS system was done via use of a Rando Phantom. A target volume of 2 x 2 x 2 cm was drilled into the phantom head. An ion chamber and thermoluminescence dosimetric chips (TLDs) were implanted in the target. A simulated treatment course consisting of 5 stereotactic radiotherapy fractions (300 cGy, 30 mm collimator) was delivered to the phantom head. A total of 27 patients who could not tolerate the GTC frame were treated using the GLAD-X/LS system. A total of 35 isocenters were used; the median number of treatment fractions was eight. Reproducibility of the x, y, and z coordinates was examined and correlated to the same determined using orthogonal port films. Relocation accuracy and reproducibility were further assessed comparing the x, y, and z coordinates of the target center with multiplanar reconstructed coronal and sagittal images. Patient tolerance of the device was also evaluated daily throughout the treatment. RESULTS: The measured TLD and ion chamber doses were within 3% of the prescribed dose at the isocenter. The same dose accuracy was also found at incremental distances of 5 mm, 10 mm, and 15 mm from the isocenter. All patients tolerated the treatment and the device well. Six patients experienced mild ear canal pain, and softer or smaller earpieces were substituted. The mean relocation accuracy was 1.5 mm +/- 0.8. CONCLUSIONS: The GLAD-X/LS system has excellent accuracy and reproducibility with the mean relocation accuracy of 1.5 mm +/- 0.8. The device is well-tolerated by patients, with no significant complications. Larger scale studies are necessary before routine use can be recommended for the administration of FSRT.

Hyperfractionated radiotherapy and paclitaxel for locally advanced/unresectable pancreatic cancer.

PURPOSE: To determine prospectively the maximal tolerated dose and potential antitumor activity of weekly paclitaxel with concurrent hyperfractionated radiotherapy in patients with locally advanced and/or unresectable pancreatic cancer. METHODS AND MATERIALS: We embarked on Phase I-II study of hyperfractionated radiotherapy using a concomitant in-field boost to a total dose of 63.80 Gy in 6 weeks at 1.1 Gy/fraction. Paclitaxel was administered weekly on Days 1, 8, 15, 22, 29, and 36 as a 3-h infusion. Paclitaxel doses were escalated from 20 mg/m(2)/wk to 70 mg/m(2)/wk. Twenty patients were studied, 14 women and 6 men (mean age 64 years). Some patients presented with one or more symptoms. Obstructive jaundice was the main presenting symptom in 10 patients and epigastric pain in 14. All patients had unresectable histologically proven adenocarcinoma of the pancreas (15 head, 4 body, and 1 tail). Reasons for unresectability were involvement of the portal vein, and/or superior mesenteric artery (n = 14), paraaortic nodes (n = 8), and medically inoperable (n = 1). Fourteen patients underwent a biliary bypass procedure before treatment (four endoscopic stenting, five choledochojejunostomy, and five cholecystojejunostomy). The follow-up period ranged from 14 to 66 months (median 44). RESULTS: The dose-limiting toxicity was observed at 70 mg/m(2)/wk. Grade IV Radiation Therapy Oncology Group late GI toxicity was seen in 1 patient in the form of duodenal stricture and hemorrhage. Grade II gastrointestinal adverse effects occurred in 13 patients and Grade 3 in 1 patient. No neurologic morbidity was encountered. Eight patients required cytokine support for Grade 2 and 3 neutropenia. The treatment course was delivered within the planned time in 80% of the patients. Complete relief of pain occurred in 10 of 14 patients. The CA 19-9 level was either stable or decreasing in 12 of 15 patients. Of 17 assessable patients, stable disease was seen in 10, regression in 2, a partial response in 3, and a complete response in 2. CONCLUSION: The use of hyperfractionated radiotherapy to a dose of 63.80 Gy with concomitant weekly paclitaxel is tolerated. The maximal tolerated dose of paclitaxel for this study was 60 mg/m(2)/wk. The preliminary objective responses denote activity of the regimen. We recommend testing this regimen in larger scale studies.

Recurrent cholangiocarcinoma: negative autopsy results after aggressive management.

Cholangiocarcinoma is one of the most common malignancies of the biliary tree. Most cases are perihilar. Cholangiocarcinoma usually has an indolent, slowly progressive course and is associated with a high mortality rate. In this article, we discuss the management of perihilar cholangiocarcinoma by radiation therapy and chemotherapy in a case in which no recurrence was found on autopsy.

Hyperbaric oxygen therapy for the treatment of radiation-induced sequelae in children. The University of Pennsylvania experience.

BACKGROUND: The role of hyperbaric oxygen (HBO) therapy in the treatment of radiation-related sequelae in adults is well known. In contrast, its role in the management of radiation-related sequelae in children has not been well studied. In an effort to define its value better, the authors reviewed the University of Pennsylvania experience and hereby report the results of their analysis. METHODS: Between 1989 and 1994, ten patients who underwent radiation therapy for cancer as children were referred for HBO therapy. Six patients underwent HBO therapy as a prophylactic measure prior to maxillofacial procedures; dental extractions and/or root canals (four patients), bilateral coronoidectomies for mandibular ankylosis (one patient), and wound dehiscence (one patient). Therapeutic HBO was administered to four other patients; one patient for vasculitis resulting in acute seventh cranial nerve palsy and the other three after sequestrectomy for osteoradionecrosis (mastoid bone, temporal bone, and sacrum, respectively). Osteoradionecrosis was diagnosed both radiologically and histologically after exclusion of tumor recurrence. The number of treatments ranged between 9-40 "dives" (median, 30 dives). Treatments were given once daily at 2 atmosphere absolutes for 2 hours each. Adjunctive therapy in the form of debridement, antibiotics, and placement of tympanotomy tubes was administered to two patients. Ages at HBO treatment ranged from 3.5 to 26 years (median, 14 years). Six patients were male and four were female. The most commonly irradiated site was the head and neck region (eight patients; brain stem gliomas [one], posterior fossa primitive neuroectodermal tumor [one], rhabdomyosarcomas [three], nasopharyngeal cancer [one], carcinoma of the parotid gland [one], and Hodgkin's disease [one]). The remaining two patients received radiation therapy for pelvic tumors [Ewings's sarcoma and rhabdomyosarcoma). Radiation doses ranged between 4000 and 6660 centigray (cGy) (median, 5500 cGy). The interval between the end of radiation therapy and HBO treatment ranged between 2 months and 11 years (median, 15 years). The median follow-up interval after HBO therapy was 2.5 years (range, 2 months-4 years). RESULTS: Except for two patients who had initial anxiety, nausea, and vomiting, the HBO treatments were well tolerated. In all but one patient, the outcome was excellent. In the six patients who had prophylactic HBO, all continued to demonstrate complete healing of their orthodontal scars at last follow-up. In the four patients who received HBO as a therapeutic modality, all 4 had documented disappearance of signs and symptoms of radionecrosis and two patients demonstrated new bone growth on follow-up computed tomography scan. One patient with vasculitis and seventh cranial nerve palsy had transient improvement of hearing; however, subsequent audiograms returned to baseline. CONCLUSIONS: The use of hyperbaric oxygen for children with radiation-induced bone and soft tissue complications is safe and results in few significant adverse effects. It is a potentially valuable tool both in the prevention and treatment of radiation-related complications.