PurposeRadiation therapy (RT) is a common treatment for benign diseases in Germany. Because the treatment concepts are inconsistent, we conducted this pattern-of-care study on behalf of the German Cooperative Group on Benign Diseases to evaluate treatment standards in Germany.Methods and materialsQuestionnaires were mailed to all radiation therapy facilities in Germany. We assessed the treatment equipment, annual number of patients, treatment indications, and, in particular, treatment strategies in patients with benign diseases in 2014.ResultsWe evaluated questionnaires returned by 116 participating institutions, of which 41 were ambulatory health care centers, 28 were private institutions, 27 were community hospitals, and 20 were university hospitals. On average, 2 linac accelerators and 2 megavoltage units were available in each institution. In 2014, a total of 36,830 patients were treated for benign diseases: 16,989 for degenerative diseases (peritendinitis humeroscapularis n = 2691; epicondylitis humeri n = 3788; heel spur n = 10,510); 14,936 for osteoarthritis (coxarthrosis n = 2230; gonarthrosis n = 2623; omarthrosis n = 2691; rhizarthrosis n = 2440; polyarthrosis n = 2297; others n = 2655); 1563 for hyperproliferative diseases (morbus Dupuytren n = 960; morbus Ledderhose n = 441; keloids n = 139; pterygium of the conjunctiva n = 3; other hyperproliferative diseases n = 20); 2440 for functional disorders (gynecomastia n = 843; Graves' disease n = 205; lymphatic fistula n = 178; heterotopic ossification prophylaxis n = 1214); 859 for stereotactic RT in the central nervous system (arteriovenous malformation n = 53; meningioma n = 425; acoustic neuroma n = 201; pituitary adenoma n = 131; others n = 49), and 43 for rare indications (pigmented villonodular synovitis n = 20 or vertebral hemangioma n = 23). The mean whole dose was <10 Gy in the treatment of degenerative disorders, 25 Gy for hyperproliferative diseases, 15 Gy for functional disorders, and <50 Gy for stereotactic RT.ConclusionsIn 2014, RT had an important role in the treatment of benign diseases. Because treatment concepts are inherent, we recommend treatment based on the guidelines written by the German Cooperative Group on Benign Diseases.
Hypoglycemia and hyperammonemia (>500µg/dl) in a newborn led to the suspicion of THAN (transient hyperammonemia of the newborn). Subsequently, hypoglycemic and salt losing episodes with low cortisol (<0,1 ug/dl) and ACTH (<0,16 pg/ml) levels pointed to ACTH deficiency. Genetic analysis showed a homozygous mutation c.302G>A for p.(Trp101*) in the TBX19 gene (a positive regulator of the transcription of POMC and the terminal differentiation of the corticotrophs), generating a premature stop codon. This mutation has been described only once and very recently by Abali et al (Hormones 18:229; 2019) in a 4 year old girl, but unlike our patients, this girl was obviously unaffected during her neonatal period. All other pituitary axes in our patient were normal, thus congenital isolated ACTH deficiency was the final diagnosis. Hyperammonemia resolved spontaneously and the suspected diagnosis of THAN could be dismissed. Hyperammonemia had probably been due to metabolic stress.After 16 months, a younger brother was born and showed hypoglycemia, hypotension and respiratory infection during his neonatal period. Cortisol and ACTH levels were also very low, thereafter, the same TBX19 mutation was detected.Both brothers were successfully treated with oral hydrocortisone substitution (6–10 mg/sqm/day q8 with increases during stress) and thrive well, except for several infections of the upper respiratory tract in the younger brother. In summary, we report the very rare condition of familial isolated congenital ACTH deficiency with a mutation of TBX19 that has never been described in newborns. Initial presentation may be accompanied by confounding pathological lab findings, while genetic analysis together with extremely low ACTH and cortisol levels confirm the correct diagnosis.
The purpose of this study is to evaluate the treatment results and toxicity of patients with breast lymphomas treated in modern era. Materials/Methods: We reviewed consecutive 23 patients with early stage breast lymphoma treated by radiation therapy with/without pharmaceutical therapy between 1997 and 2013 at single institution. All patients were histologically proven with malignant lymphoma. Information of patient characteristics, detailed radiation treatment, toxicities, relapses and salvages treatment, and outcomes were collected with chart review. Results: The characteristics of patients were following; median age 56 years (range, 17-80 years old), female:23, clinical stage I:14 II:8, pathology DLBCL:20 MZBCL:2 ALCL:1. As an initial treatment, RCHOP was prescribed for 13 DLBCL patients, CHOP4/6 was for 4, and EPOCH was for 2. Of them, 3 patients received intra-thecal MTX. Two patients with MZBCL were treated with radiation therapy alone without any chemotherapy. The median radiation dose was 40 Gy (range, 30-54). The IFRT was delivered to the whole breast for stage I patients and EFRT was delivered to the whole breast and regional lymph nodes for stage II patients. The median follow up time was 66 months (range 3-180). Three patients died with lymphoma, 1 died of other disorder, and 19 patients are alive without any evidence of relapse. The local control was achieved in all 23 patients. One patient developed marginal relapse close to the IFRT field. There were 4 relapses documented outside the irradiated fields; 2 distant lymph nodes, 1 bone and 1 bone marrow respectively. Two systemic DLBCL relapse of 4 were developed 15 years after initial treatment. There was no CNS relapse in this series. No toxicities greater than grade 2 were observed during treatment and over follow up time. Conclusion: The radiation therapy for breast lymphomas provides excellent local control with low toxicities. The CNS prophylaxis might be safely omitted for low IPI patients with breast lymphomas. We have to pay attention to the delayed relapsed 10 years or later.
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