Sinonasal phosphaturic mesenchymal tumour: radiation oncologist's perspective.

Tumour-induced osteomalacia is a rare cause of osteomalacia, the majority of which is of mesenchymal origin. Oncogenic osteomalacia is a potentially curable condition caused by phosphaturic mesenchymal tumours. We present the case of a woman in her 30s with a sinonasal phosphaturic mesenchymal tumour, treated with surgical excision followed by adjuvant intensity-modulated radiotherapy and subsequent adjuvant chemotherapy. The patient experienced minimal adverse effects during radiation. There was good local control and cosmetic outcomes with no radiation-related toxicity at a follow-up period of 32 months.

Malignant phosphaturic mesenchymal tumor, mixed connective tissue variant of the tongue.

The majority of the oncogenic osteomalacia-associated mesenchymal tumors are considered to belong to the category of phosphaturic mesenchymal tumors, mixed connective tissue (PMTMCT) variant, of which malignant cases are very rare. Here we report a case of a recurrent malignant PMTMCT variant which arose in the tongue. The patient was treated with surgery at an initial treatment and the first recurrence. In accordance with the tumor recurrence and resection, the hypophosphatemia progressed and improved. However, hypophosphatemia did not progress after receiving radiation therapy at the second recurrence even though the recurrent tumor gradually increased its size. These results suggest clinical feature of malignant PMTMCT could be changed by radiation therapy. Thus, this report could add an insight to the nature of PMTMCT.

Intermandibular malignant mesenchymoma in a crossbreed dog.

A 12-year-old German shepherd crossbreed dog was presented with a submandibular mass that was initially diagnosed as myxosarcoma on incisional biopsy. Chest radiographs were taken for staging, and magnetic resonance imaging was performed to assess the feasibility of cytoreductive surgery before adjuvant radiotherapy. The dog underwent debulking surgery, and histology permitted reclassification of the tumour as a malignant mesenchymoma (with myxosarcomatous and osteosarcomatous differentiation). The dog was subsequently treated with four fractions of radiotherapy given at seven-day intervals and three doses of carboplatin. The dog remained stable following therapy until its condition acutely deteriorated, and it was euthanased 153 days after surgery. On postmortem examination, there were no signs of local tumour recurrence, but metastases were observed both in the thorax and in the abdomen.

Radiation-induced chorioretinal degeneration: a clinicopathological report of three cases.

BACKGROUND: With the shift in radiotherapy toward the posterior segment and with the use of lead screens to shield the anterior segment, posterior segment lesions have become more readily recognized. The purpose of this study is to highlight the effects of ionizing radiation on the choroid and to demonstrate how this can result in visual loss, particularly if the macula is involved. METHODS: Histopathological study of three enucleated eyes of three patients who had received ionizing radiation: a 27-year-old woman who had received radiation as a child for a hemangioma of the left side of the face, a 16-year-old girl who had received radiation at age 11 years for a malignant mesenchymoma of the right maxilla, and a 4-year-old girl who had received radiation at age 1 year for a retinoblastoma of the right eye. RESULTS: Histopathological examination of the three globes showed extensive chorioretinal degeneration, among other ocular findings. In all cases the fellow eye did not show similar chorioretinal lesions. As all three patients were relatively young, the degree of chorioretinal degeneration was considered to be secondary to radiation treatment. INTERPRETATION: Vascular damage from ionizing radiation is not limited to the retina. It can also affect the choroid in the form of chorioretinal degeneration. Since most of the intraocular circulation arises from the uveal vessels, chorioretinal degenerative lesions may be extensive and may even involve the macula.

[Solitary fibrous thoracic wall tumor. Progression with percutaneous radiotherapy]. / Solitärer fibröser Thoraxwandtumor. Progredienz unter perkutaner Radiatio.

HISTORY AND CLINICAL FINDINGS: A 81-year-old patient free of pain was referred to the university hospital for further evaluation and therapy of tumour masses in the right thorax. Clinical examination revealed dullness to percussion and reduced breathing in the right lower lung. INVESTIGATIONS: Computed tomography showed an enlarged solid tumour mass attached to the thoracic cavity and pleural effusion on the right side. Quantification of pulmonary perfusion presented significant defects in the right upper and middle lobe. DIAGNOSIS, TREATMENT AND COURSE: The pulmonary masses were biopsied under CT-guidance. Biopsy and immunohistochemical findings proved a malignant solitary fibrous tumour of the chest wall, a mesenchymal tumour of its own entity. Because of pain in the right arm and because of missing other reliable therapeutic options a palliative irradiation was performed. The tumour did increase in size due to radiotherapy and a severe right ventricular heart failure occurred. The patient died 5 months after diagnosis has been made. Autopsy revealed a transition of tumour cells to sarcomatic growth. CONCLUSION: In our case we conclude an accelerated progression of the solitary fibrous chest wall tumour in the course of irradiation. Whether the development of sarcomatic growth occurred as a result of radiotherapy remains speculative.

Stromal sarcoma of the breast: a case report.

Malignant mesenchymal tumours of the breast are rare neoplasms. Their incidence varies from 1%-3% of all malignant breast tumours (1). Stromal sarcoma of the breast is an extremely uncommon tumour (2). We present a rare case of advanced stromal sarcoma of the breast successfully managed with radical surgery and radiotherapy.

Embryonal sarcoma of the liver in an adult treated with preoperative chemotherapy, radiation therapy, and hepatic lobectomy.

A rare case of embryonal sarcoma of the liver in a 28-year-old man is reported. The patient was treated preoperatively with a combination of chemotherapy and radiation therapy. Complete surgical resection, 4.5 months after diagnosis, consisted of a left hepatic lobectomy. No viable tumor was found in the operative specimen. The patient was disease-free 20 months postoperatively.

A randomized clinical trial of adjuvant adriamycin in uterine sarcomas: a Gynecologic Oncology Group Study.

After hysterectomy, 156 evaluable patients with stage I (limited to the corpus) or stage II (limited to the corpus and cervix) uterine sarcomas were randomly assigned to adjuvant chemotherapy with Adriamycin (Adria Laboratories, Columbus, Ohio) for six months or to no further treatment. Pelvic irradiation (external or intracavitary) was optional before randomization. Of 75 patients receiving Adriamycin, 31 have suffered recurrences compared with 43 of 81 receiving no adjuvant chemotherapy. This difference is not statistically significant. Moreover, there is no difference in progression-free interval or survival. The optional radiotherapy did not influence the outcome although there was a suggestion that vaginal recurrence was decreased by pelvic radiotherapy. The recurrence rates in specific cell types (leiomyosarcoma, homologous mixed mesodermal sarcoma, or heterologous mixed mesodermal sarcoma) were not significantly different although the pattern of recurrence differed, with pulmonary metastases being more common in leiomyosarcoma and extrapulmonary recurrence being more common in mixed mesodermal sarcoma. The outcome with respect to chemotherapy was not altered even after adjusting for maldistribution of cases. Thus, we could not show a benefit for this dose schedule of Adriamycin as adjuvant treatment for uterine sarcomas.

Primary retroperitoneal sarcomas. A review of 36 cases.

A retrospective study of 36 patients with primary retroperitoneal sarcomas treated at The Norwegian Radium Hospital is presented. The median age at presentation was 57 years. The most common presenting symptom was abdominal pain. Leiomyosarcoma and liposarcoma were the most common histologic subtypes. The median survival in the whole series was 25 months. Patients with completely resected tumors had a longer median survival (59 months) than patients with incomplete resection (16 months) but the difference was not statistically significant. The malignancy grade seemed to be the most important prognostic factor and patients with low grade tumors had a significantly better outlook than those with high grade tumors.

Kidney damage by ionizing radiation.

The report describes a case of radiation damage to a kidney due to radiotherapy of a sarcoma of the muscles of the back. The characteristic clinical, pathological and histological findings and the results of X-ray investigation are presented in detail.

[Radiotherapeutic methods in the management of ovarian cancer (author's transl)]. / Methodik der strahlentherapeutischen Massnahmen beim Ovarialkarzinom.

A report is given of the therapeutic strategy and the stage-adjusted methods of irradiation in the treatment of ovarian cancer. The influence of radical surgery, irradiation and chemotherapy on survival rates is discussed and the results achieved in this unit, especially in patients admitted at a more advanced tumour stage, are presented.

[Primary radiation therapy of sarcoma of the uterus (author's transl)]. / Die primäre Radiotherapie des Uterussarkoms.

We report 24 cases of sarcoma of the corpus uteri treated by sole irradiation. The 5-year-survival-rate was 20,8%. The prognosis seems to be poor in this group treated by sole irradiation. Only inoperable cases of sarcoma of the corpus uteri ought to be treated by radiotherapy alone.

Cerebral necrosis following radiotherapy of extracranial neoplasms.

We have examined 6 patients with delayed cerebral necrosis following irradiation of extracranial neoplasms. Four of the 6 patients received 1,760 rets (or less) tumor dose. The initial symptoms attributable to radiation necrosis appeared 4 to 31 months after irradiation and were those of a focal supratentorial mass. Cerebral angiography delineated an avascular frontal or temporal lesion in all 6 patients; in 1 case a magnification study revealed narrowing, irregularity, and occlusion of small cortical vessels. Four of our 6 patients underwent craniotomy with partial or complete surgical extirpation of necrotic brain tissue. Two operated patients are alive and without disabling neurological symptoms 30 and 25 months, respectively, after the operation. The characteristic neuropathological features of delayed radiation necrosis of brain suggest that vascular injury rather than neuronal or glial damage is of primary pathogenetic significance.

Embryonal rhabdomyosarcoma of head and neck in children. Correlation of stage, radiation dose, local control, and survival.

Nineteen consecutive children are analyzed according to clinical stay, radiation dose (NSD), local control, and survival. The majority received 1600 to 1750 rets and courses of actinomycin during their radiation treatments. The favorable sites were the orbit, facial soft tissue, and the larynx. The primary site control rate was 89%, and the metastatic neck control rate was 80%. The 2-year survival was 70%, and the 5-year survival was 67%. Five children are alive and well 12 to 15 years after irradiation. Late sequelae are hypoplasia of the orbit and maxillary sinus.