ABVD chemotherapy with reduced radiation therapy rates in children, adolescents and young adults with all stages of Hodgkin lymphoma.

Background: We adopted ABVD chemotherapy with risk-adapted radiation therapy (RT) as first-line therapy for children, adolescents and young adults with Hodgkin lymphoma (HL) in British Columbia in 2004. Patients and methods: Patients ≤ 25 years diagnosed from 2004 to 2013 with all stages of HL who received ABVD as initial therapy were included. Results: Among 55 children (age < 18 year) and 154 young adults (18-25 year), there were no significant differences among age groups for sex, histologic subtype, tumour bulk, B symptoms, prognostic risk groups or treatment received. The rates of complete response, partial response and progressive disease were 84%, 7% and 10% for children and 95%, 4% and 1% for young adults (P=0.01), respectively. Treatment failures in children all occurred within one year of completion, while 8/21 (38%) relapses in young adults occurred later (P=0.04). With a median follow-up of 66 months the 5-year progression-free (PFS) and overall survival (OS) were 85 ± 3% and 97 ± 1%, respectively. For limited stage disease, PFS was 90 ± 7% for children and 93 ± 3% for young adults (P=0.65); OS was 100% for both. For advanced stage patients, PFS and OS were also similar for the children and young adults (77 ± 7% versus 81 ± 4%; P=0.38 and OS 90 ± 6% versus 97 ± 2%; P=0.17). The rate of consolidative RT was low (21%) and did not differ between age groups. Conclusion: ABVD is an effective treatment in children, adolescents and young adults with HL. Children were less likely to achieve complete response and demonstrated earlier relapses compared to young adults. RT may be omitted for the majority of patients while maintaining excellent 5-year OS.

Isolated adrenocorticotrophin deficiency and empty sella associated with hypothyroidism.

A case of isolated ACTH deficiency is described. The patient had an empty sella and longstanding radio-iodine induced hypothyroidism. The association between isolated ACTH deficiency, empty sella and hypothyroidism is reviewed. It has not previously been recognized that the commonest endocrine association of isolated ACTH deficiency is primary thyroid failure. ACTH secretion does not recover following thyroxine replacement treatment.

Computed tomography of retroperitoneal and mesenteric sarcomas: a pictorial essay.

Retroperitoneal and mesenteric sarcomas are rare tumours that often do not present until they are very large. Computed tomography (CT) is helpful for diagnosis and for planning therapy. This pictorial essay illustrates the spectrum of CT manifestations of these tumours that the authors have encountered and includes a review of the various aspects of clinical presentation and treatment.

Age is the major determinant of recurrence in pediatric differentiated thyroid carcinoma.

BACKGROUND: A relationship between young age and increased risk of recurrence of pediatric differentiated thyroid carcinoma has been suggested; however, no attempts have been made to assess the prognostic factors or efficacy of treatment in very young children with this malignancy. The objectives of this study were to evaluate the association of age with outcome in pediatric differentiated thyroid carcinoma and to compare the clinical, pathologic, prognostic, and treatment variables between younger and older children with this disease. PROCEDURE: A retrospective review of all patients presenting to the British Columbia's Children's Hospital or British Columbia Cancer Agency <17 years of age at diagnosis with differentiated thyroid carcinoma between January, 1955, and December, 1996, was completed. RESULTS: Thirty-eight patients were identified, 12 of whom were 10 years. An association between young age and extrathyroidal tumor invasion was identified (P = 0.016); however, the latter factor did not independently predict outcome. There was a trend for suppressive doses of thyroid hormone to improve outcome, particularly with increasing age at diagnosis, but this was not statistically significant. CONCLUSIONS: Age is the major determinant of recurrence in pediatric differentiated thyroid carcinoma. The results suggest different tumor biology in young children requiring novel approaches to therapy to decrease recurrence rates.

Local excision without radiation for high-grade soft-tissue sarcoma of the extremity and superficial trunk.

Purpose. Limb-sparing surgery combined with radiation treatment has become the accepted treatment for patients with high-grade soft-tissue sarcoma. Adjuvant radiation was not routinely used at this institution for patients with clear margins after surgery.This retrospective review analyses the outcome of this group of patients.Patients and methods. Patients studied were referred from 1984 to 1995, were over 16 years of age, were diagnosed with primary high-grade soft-tissue sarcoma of the extremity or superficial trunk, had clear margins after excision and did not receive radiation as a part of their initial treatment. A total of 46 patients were identified.Results. At 5 years, the local control rate was 87%, disease-specific survival was 75% and overall survival was 68%. Of the 6 local recurrences, 3 were located in the buttock (from a total of 7 patients with primary tumours of the buttock), 3 had a primary size of >/= 10 cm (from a total of 8 primary tumours of that size) and all were deep tumours.Discussion. Our data, and those from other reports, suggest that in carefully selected patients appropriate surgery alone results in acceptable local control and survival, and that the morbidity of radiation can be avoided.