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

AIM: To describe the pattern of paediatric Rhabdomyosarcomas (RMS) and Non-Rhabdomyosarcomas (NRMS) with emphasis on the indices that affect survival outcomes. METHODS: We reviewed all patients with histologically confirmed RMS and NRMS in the Departments of Pathology and Paediatrics, University College Hospital (UCH), Ibadan, Nigeria; in children aged 0-14 years. The study period was January 1991 to December 2016. Information obtained included age, gender, morphology and site of the tumours. The tumour grade and pathologic/clinical staging of all patients were also obtained and verified by the clinical records. Tumour grading was carried out using the Fédération Nationale des Centres de Lutte Contre le Cancer (FNCLCC) Sarcoma group grading system and staging was done using TNM. Follow up, survival information and final outcome were retrieved. RESULTS: The 104 patients included in the study had almost equal male-to-female ratio, age ranged between 5 months and 14 years (median 8.2 years). Rhabdomyosarcoma had mean age of 5.6 (±3.8) years while that of NRMS was 9.2(±4.1) years. Overall, the modal age group was 5-9 years. Rhabdomyosarcoma was the commonest histological type (76%), undifferentiated sarcomas (6.7%), fibrosarcoma (3.8%) and 2.9% each for synovial sarcoma and dermatofibrosarcoma protuberans. The common primary sites were the head and neck (including the orbit) 49 (47.1%), and the abdominopelvic 26 (25%) regions. Majority (89%) had histologic grade 3 at presentation. Seventy per cent and 64% of patients with RMS and NRMS, respectively, had high stage tumour at presentation. Median survival for all patients with Rhabdomyosarcoma was 45 weeks with a 1-year survival of 43% and 2-year survival of 25%. Non-RMS (Dermatofibrosarcoma protuberans and Solitary fibrous tumours) had survival of over 4 year's duration. CONCLUSION: Majority of our patients presented at a late stage with histologic high grade which confers poor prognosis and reduced chances for good overall survival outcome.

P57kip2 immunohistochemical marker as a diagnostic tool for cases of hydatidiform moles in a tertiary health facility in Southwestern Nigeria.

BACKGROUND: Hydatidiform mole (HM) is the most common gestational trophoblastic disease. P57kip2 has been reported to be helpful in differentiating between partial and complete HMs. OBJECTIVES: The study aims to evaluate the P57kip2 immunohistochemical (IHC) marker as a useful ancillary investigation to differentiate complete hydatidiform mole (CHM) from partial hydatidiform mole (PHM). MATERIALS AND METHODS: A retrospective study of all histologically diagnosed HM cases over a 20 year period was undertaken. Clinicopathological parameters were extracted from the surgical day book and medical record archives. Archival haematoxylin- and eosin-stained slides and formalin-fixed paraffin-embedded tissue blocks of all cases of HM diagnosed within the study period were retrieved and reviewed. Cases of HM were reclassified using the P57kip2 IHC marker. The data obtained were analysed using the SPSS version 23. RESULTS: One hundred cases of HMs were studied. CHM accounted for 68%, while PHM accounted for the remaining 32%. The incidence of HM was 2.98 cases per 1000 deliveries. The ratio of CHM to PHM was found to be 2.1:1. Seventy-two per cent of the cases were diagnosed in the first trimester, while the remaining 28% were diagnosed in the second trimester of pregnancy. Based on the P57kip2 IHC staining pattern, HM cases were finally reclassified into 68 cases of CHM and 32 cases of PHM. The age range for all the HM cases was 18-50 years with the majority of the cases seen in the third and fourth decades of life. CONCLUSION: P57kip2 could be useful as an ancillary investigation in confirming the diagnosis of CHM and differentiating it from PHM, particularly in difficult and challenging cases.

Congenital infantile digital fibromatosis: a case report and review of the literature.

Infantile digital fibromatosis (IDF), also called inclusion body fibromatosis is an uncommon benign tumour occurring in the digits of young children. In about a third of cases, it is congenital and the diagnosis is based on the presence of peculiar intracytoplasmic inclusions on histology. Recurrence rate post-surgery is high. However, spontaneous regression has been reported. We present a case of a 5-month-old infant who had excision of a right second toe mass, which has been present from birth. Histological examination revealed this to be infantile digital fibromatosis. To the best of our knowledge, no report of this has been made in Nigeria. It is important that this diagnosis be entertained in young children with masses on the digits as this will influence the management instituted.

Gastrointestinal Stromal Tumours (GIST): A Review of Cases from Nigeria.

PURPOSE: Gastrointestinal stromal tumours (GIST) are rare and studies on GIST in Nigeria are extremely uncommon. This study aims to achieve a comprehensive systematic review of the cases of this tumour in Nigerians. METHODS: A systematic search of all available literature on GIST published from Nigeria between January 2000 and December 2018 was done and reviewed. Simple descriptive data on all the cases are presented. RESULTS: The search yielded 15 publications but 13 publications with a total of 67 patients were analysed. The other two studies centred on imatinib therapy and overall survival, and molecular characteristics respectively and were therefore reviewed independently. Age at diagnosis ranged from 9 to 75 years, with mean age being in the 50s and 60s across most studies. There is no gender disparity. Anatomical location of primary tumours showed gastric location as the most frequent (61%) followed by large intestine accounting for 15%, the small intestine (9%) and other locations (15%). Tumour size ranged from 5-39.5 cm. Over 85% of patients had tumour size greater than 10 cm at presentation, hence fell into intermediate or high-risk group irrespective of location of tumour. The overall survival of a cohort of 27 patients that had imatinib therapy was 69.5% after 4 years of follow-up. KIT exon 11 mutations were the only mutations detected from a small cohort of 12 patients. CONCLUSIONS: Characteristics of GIST in Nigerians are fairly similar to other parts of the world. However, most of our patients present with large masses which are of poor prognostic characteristics.

Parathyroid Adenoma with Unusual Presentations of Rib Bone and Thoracic Vertebrae Fractures in a Premenopausal Female in Ibadan, Nigeria.

Parathyroid adenoma is the most common cause of primary hyperthyroidism which leads to abnormal calcium homeostasis, hypercalcemia, and reduction in bone density. A 37-year-old female referred from a private health facility with a 1-year history of upper back swelling and pain. The pain was worse when sitting down for long periods and with movement and relieved by rest. There was no antecedent history of trauma, but the patient had noticed poor appetite and weight loss. There were no constipation, no abdominal discomfort, and no symptom suggestive of hyperthyroidism or hypothyroidism. General physical examination revealed kyphoscoliosis, and vital signs were within normal limits. Spine X-ray showed features of cervical spondylosis. Computed tomography (CT) scan and magnetic resonance imaging showed pathologic fractures of the right 9 thrib, anterior wedge compression, and reduction of T4 vertebrae with other abnormalities at T4-T5, T5-T6, T7-T8, T10-T11, and L4-L5 vertebrae. Bone marrow aspiration and serum electrophoresis were within normal limits. Serum calcium showed hypercalcemia. A CT scan of the neck was done which showed features of a right superior parathyroid adenoma. Blood count, other serum electrolytes, and thyroid function tests were all normal. A parathyroidectomy with right thyroid lobectomy was done. Histopathological examination of the resected parathyroid gland showed a diagnosis of parathyroid adenoma. A high index of suspicion is needed to diagnose this unusual presentation of parathyroid adenoma. Radiological imaging is an important tool for early diagnosis.

Correction to: Pattern and Significance of Tumour Budding in Colorectal Carcinomas Using ITBCC Guidelines: a Low Resource Setting Practice Observation.

The original version of this article unfortunately contained a mistake. In Table 2, the number 36 under "N" should be 96 and "Tumour size in cm (range)" should read "Tumour size in cm."

Pattern and Significance of Tumour Budding in Colorectal Carcinomas Using ITBCC Guidelines: a Low Resource Setting Practice Observation.

PURPOSE: To determine the pattern and significance of tumour budding among colorectal carcinoma (CRC) Nigerian patients using the 2016 International Tumour Budding Consensus Conference (ITBCC) guidelines. METHODS: H&E-stained slides of resected CRC at the University College Hospital and a private laboratory, both in Ibadan, Nigeria, from January 2008 to December 2017 were reviewed. Patient age, gender, tumour size and location were obtained from the surgical pathology records. Tumours were graded and staged according to the 2010 WHO and the 2017 UICC protocols, respectively. Tumour budding was determined at × 20 objective lens magnification with a 20-mm eyepiece field number diameter. Descriptive, Mann-Whitney U and chi-square test statistics were applied using SPSS 20; p < 0.05 was considered significant. RESULTS: Ninety-six cases were included in this study. Fifty-one (53.1%) showed tumour budding. Tumour bud count was low (0-4) in 66 (68.8%), intermediate (5-9) in 12 (12.5%) and high (≥ 10) in 18 (18.8%) tumours. Four tumours had pT1 stage, 35 pT2, 37 pT3 and 20 pT4. Forty-three (44.8%) tumours were lymph node-positive, and 10 (10.4%) had metastasis. Patients' age and tumour size distribution were similar in the tumour budding and non-budding groups (52.4 ± 17.1/58.5 ± 13.9 years and 6.6 ± 2.9/6.6 ± 2.8 cm, respectively). There was significant association between tumour budding and tumour grade (p < 0.008), pT stage (p < 0.000), lymphovascular permeation (p < 0.000), perineural invasion (p < 0.003) and nodal status (p < 0.034), but not with gender (p = 0.588), metastasis (p = 0.327) and TNM group-stage (p = 0.062). CONCLUSION: Tumour budding frequency is high among our CRC patients and is associated with poorer prognostic factors.