Pediatric Soft Tissue and Bone Sarcomas in Tanzania: Epidemiology and Clinical Features

Abstract

PURPOSE Pediatric sarcomas represent an important group of childhood tumors that require treatment at Muhimbili National Hospital (MNH), the largest pediatric oncology center in Tanzania. Treatment is often adapted from established childhood protocols validated in clinical trials from the United States and the United Kingdom. There are no studies describing the types of pediatric sarcomas most commonly seen in Tanzania to understand similarities and disparities with other countries and which sarcomas to prioritize in adapting treatment protocols. The objective of this study was to establish a baseline of the epidemiologic and clinical features of pediatric sarcomas diagnosed at MNH. METHODS Information was collected on clinical and tumor features of all children seen at MNH pediatric oncology unit between 2011 and 2016 with a confirmed histologic diagnosis of either bone or soft tissue sarcoma (STS). RESULTS A total of 135 cases were analyzed; 89 (66%) were STS and 46 (34%) were bone sarcomas. There was a slight female predominance (n = 69; 51%), and the mean age (SD) of patients was 6.3 (5.1) years. Greater than 90% (n = 123) of the cases presented with a painless swelling. The commonest STS, accounting for almost three-fourths of the cases (n = 66) was rhabdomyosarcoma (RMS), with embryonal subtype being the most common RMS (n = 49; 74%). Osteosarcoma was the most common bone sarcoma, accounting for greater than 80% (n = 40) of the cases. Ewing sarcoma accounted for less than 15% (n = 6). Most of the patients presented with stage IV disease (n = 57; 87%) and lung was the commonest metastatic site. CONCLUSION To our knowledge, this report is the first study documenting the epidemiologic and clinical features of pediatric sarcomas in a modern Tanzanian pediatric hospital. Embryonal RMS and osteosarcomas should be prioritized for adapting treatment protocols from other countries.

Paediatric soft tissue sarcomas in a resource constraint setting: Grade and stage at presentation and at oncologic intervention are usually of poor prognostic characteristics

“…8,21,25,27,28 In this study,the predominance of embryonal and alveolar RMS supports the literature on childhood occurrence of these subtypes of sarcomas while the very few cases of pleomorphic RMS in this study aligns with existing literature as well. 23,29 Other adult-type STS seen in this study differ somewhat in spectrum from those seen elsewhere. 27 Chondrosarcoma, epitheloid and clear cell sarcoma reported by Tinkle et al in the United states 27 were not seen in our study.…”

Clinico-pathological profile of paediatric head and neck cancers in Tanzania: Findings from the country’s largest tertiary hospital

“…This study found several sites to be affected predominantly in males similar to what has been found elsewhere where the orbit cervical lymph nodes and nasopharynx had male predominance. 2,12,15,23,[26][27][28][29] No sex predilection was found in terms of affection of the sinonasal region in this study but a study in Nigeria found male predominance but another from United States of America had female preponderance. Such differences may be accounted by variable epidemiological distribution of head and neck cancers.…”

“…Similar findings were reported in the study which was conducted by Khademi et al in Iran and Siwillis et al in Tanzania where the sinonasal cavity (nasal cavity plus paranasal sinuses) was predominantly affected by sarcomas. 27,30 This study had several limitations; the study was carried out in one tertiary hospital which may not be the representative of the entire country and the study being a snap shot experience over a relatively short period of time.…”

Primary intratesticular rhabdomyosarcoma in children: a case report and review of the literature

“…In 2013, the World Health Organization (WHO) report on classification of RMS includes four histological subtypes namely alveolar, embryonal, pleomorphic and sclerosing or spindle cell types of RMS [ 3 ]. It is estimated that embryonal RMS represents about 70% of all childhood RMS especially those below 10 years of age [ 4 , 5 ]. Of the head and neck regions, the orbit is the commonest site for embryonal RMS.…”

“…These results are due to multidisciplinary treatment approaches including surgery, radiotherapy and especially chemotherapy. RMS is a highly chemosensitive neoplasm, and the role of this therapeutic approach has also been clearly demonstrated in the adjuvant setting [ 5 , 10 ].…”