Paediatric intussusception in Benin city, Nigeria

Intussusception is one of the leading causes of intestinal obstruction in children. This study reports our experience in the management of intussusception in Benin City; Nigeria. This was a 10-year retrospective review of intussusception in children at the University of Benin Teaching Hospital from January; 1997 to December; 2006. Twenty four children were admitted with intussusception (M: F = 1:1.4) within the period. Only three patients (12.5) presented within 24 hours of onset of illness. Ten (41.6) presented between one and four days; seven (29.2) between five and 10 days; while four (16.6) presented between 11and14 days. Abdominal pain; irritability; blood in stools; vomiting; abdominal distension and palpablemass in various combinations were the clinical features. All the patients; except one (4); were below the age of one year. There was much delay before presentation of infantswith intussusception; with high attendant mortality

Winer's dilated pore, rare presentation in the external ear canal.

A 51-year-old female patient was admitted to our outpatient department with a discharging right ear of six months duration and not responding to aural toilet and topical antibiotics. Microscopic examination showed a collection of soggy keratin in the floor of the ear canal. Suction cleaning showed a skin defect of about 7 mm in diameter. A CT scan showed rarefaction in the bony canal. Histological diagnosis following an excision biopsy under general anaesthetic showed Winer's dilated pore. Winer's dilated pore is a hair follicle tumour arising from the pilosebaceous apparatus. Hair follicle tumours are relatively rare and their clinical appearance is commonly non-specific. In spite of their non-specific features, they show a keratotic plug grossly which when removed reveals a skin defect and may show bone erosion occasionally if the underlying structure is bone. These features can be misinterpreted for more serious lesions such as squamous cell carcinoma or basal cell carcinoma of the ear canal that can lead to pursuing a more aggressive line of management for a benign lesion. We present this rare lesion illustrating the pathological features, differential diagnosis and management.

Schwannoma with features mimicking neuroblastoma: report of two cases with immunohistochemical and ultrastructural findings.

OBJECTIVE: A study of two cases of a rare variant of benign schwannoma showing areas mimicking neuroblastoma/peripheral primitive neuroectodermal tumour (PNET). METHODS: Sections of formalin fixed, paraffin embedded specimens were studied by tinctorial stains and immunohistochemistry, and the tissue retrieved from formalin was examined by electronmicroscopy in one case. RESULTS: The tumours were small and subcutaneous. Both showed features of benign schwannoma; one had a multinodular plexiform pattern. In addition, rosette-like structures consisting of collagenous cores surrounded by small round cells or slightly larger epithelioid cells were present. Tumour cells were positive for S100 protein, Leu7, and in one case GFAP, but were negative for neurofilament protein, synaptophysin, and MIC2. Type IV collagen surrounded individual cells. Electronmicroscopy in case 2 confirmed schwannian features (lamina, processes) and failed to show features of neuroblastoma (neuroendocrine granules). CONCLUSIONS: Benign schwannomas may contain rosette-like structures mimicking neuroblastoma/PNET. The techniques used confirmed schwannian differentiation only and eliminated neuroblastoma/PNET. These uncommon variants should be recognised by practising histopathologists to avoid erroneous diagnoses and inappropriate treatment.

Atypical eumycetoma caused by Phialophora parasitica successfully treated with itraconazole and flucytosine.

Phialophora species are occasional pathogens causing subcutaneous and invasive disease. We report the first case of eumycetoma caused by P. parasitica in an otherwise healthy U.K. resident who visited India. She failed to respond to surgical excision and itraconazole, 400 mg daily, but responded to itraconazole, 400 mg daily, and flucytosine, 1 g three times daily, for 12 months. In vitro susceptibility testing predicted a response.

Renal botryomycosis.

A case of visceral botryomycosis which arose in the left kidney of a 60-year-old woman is reported. This is the fifth reported case of renal botryomycosis which, in this patient, was mistaken clinically for a renal carcinoma. The lesion was composed of confluent abscesses containing 'sulphur granule-like' lesions in which irregularly lobed aggregates of Gram-negative organisms surrounded by an eosinophilic capsule were noted. Awareness of this unusual lesion is important because of its histopathological similarity to actinomycosis.

Intra-oral lipomata in association with congenital anomalies: a variant of Mohr's syndrome?

Two cases of intra-oral lipomata occurring in association with congenital anomalies are reported. These appear to be the first such cases described, in what appears to be a variant of Mohr's syndrome.

A study of spindle cell sarcomas showing myofibroblastic differentiation.

Five diagnostically problematic spindle cell sarcomas showing invasive character, cellular pleomorphism, and high mitotic rate were studied clinically and histopathologically by conventional light microscopy, immunohistochemistry, and transmission electron microscopy. They showed varied clinical courses, with two causing death within 5 years and three showing recurrent and metastatic behavior. All lacked a clearly defined line of differentiation by conventional light microscopy. By immunohistochemistry, all were positive for vimentin and alpha-smooth muscle actin; in addition, one showed focal S-100 protein positivity, and one stained for desmin. All were cytokeratin negative. By electron microscopy, the great majority of spindle cells in all cases showed abundant rough endoplasmic reticulum and fine myofilaments with focal densities; collagen secretion granules were also found in all cases but in fewer cells. The fine structure and immunophenotype were considered consistent with myofibroblastic differentiation; these tumors, therefore, were designated as sarcomas of myofibroblasts or myofibrosarcomas. The suitability of the alternative diagnostic label of myofibroblastic or matrix-secreting variant of leiomyosarcoma is discussed. Comparisons with similar tumors documented in the literature are drawn.

Carcinoid tumour of the larynx (a case report and review of the literature).

Carcinoid tumours of the larynx are extremely rare, only 13 cases having previously been reported in the world literature. A case is reported here with the unusual complication of skin metastases. The true diagnosis is seldom made on the original biopsy, which is commonly reported as poorly differentiated carcinoma. A review of the literature is made with a discussion of the histological diagnosis and treatment modalities.

Corynebacterium parvum followed by chemotherapy (actinomycin D and DTIC) compared with chemotherapy alone for metastatic malignant melanoma.

Seventy-nine patients with Stage III widely metastatic melanoma were prospectively randomised to a 'no treatment' control group who received on tumour progression DTIC (250 mg/m2 i.v. daily X 5) and Actinomycin D 1.5 mg/m2 on Day 1. A total of six courses at 3-week intervals was given. Chemotherapy was only given on progression of disease. The other group received initially Corynebacterium parvum (2 mg/m2) every 3 weeks for a maximum of eight courses and then the same chemotherapy on evidence of progressive disease. Minimum follow up time is 3 yr. The chemotherapy response rate (control 37%, C. parvum 24%) was not statistically different nor was the effect of chemotherapy on the site of individual metastases. Radiotherapy responses for irradiated soft tissue disease again were not significantly different, between the two patient groups. No significant differences in survival (control group median, 4 months, range 1-46; C. parvum median 3 months range 1-35) were observed and only one patient is alive at 35 months. The pattern of relapse was also similar in both groups. Reduction in haematological toxicity consequent on chemotherapy was not observed in the C. parvum-treated patients. No additional benefit was observed when C. parvum was followed by DTIC and Actinomycin D chemotherapy compared with the results from the chemotherapy given alone, although C. parvum on this schedule had minimal toxicity.

Randomized study of Corynebacterium parvum adjuvant therapy following surgery for (stage II) malignant melanoma.

One hundred and fifteen patients with histologically proven primary cutaneous melanoma were entered into a randomized prospective study following surgical removal of clinically obvious regional metastases. The study started in 1977 and was completed in June 1982. Fifty-eight patients (10 with regional skin metastases and 48 with regional node metastases) were in the control group and 57 (15 regional skin, and 42 regional node metastases) received adjuvant C. parvum. Other clinical features were well balanced for each group. C. parvum, 2 mg/m2 IV was repeated eight times at three weekly intervals. The schedule was based on previous work which indicated significant increase in killer cell activity. There was no serious toxicity and 22 patients exhibited tachyphylaxis. Seventeen patients in the control group (11 tumour free) are alive at 24-80 months, and 20 patients in the C. parvum group (12 tumour free) are alive 24-76 months from randomization. No statistically significant difference (P greater than 0.05) was observed between the two patient groups for overall survival (median 26 months) or relapse free survival (median 8 months) from the date of randomization. Nor was the overall survival from the date of primary surgery (median 52 months, range 4-237) or the relapse free survival, median 28 months, range 2-225, significantly different between the two groups of patients. The relapse pattern in the two groups, and survival after relapse were no different though the response rate to actinomycin D and DTIC chemotherapy was significantly higher in the control (48 per cent) than in the C. parvum (23 per cent) group. Toxicity to chemotherapy was not significantly affected by previous C. parvum treatment. Although there were trends for better survival, in the C. parvum patient group, statistically significant activity has not been noted in this study.

Involvement of the vulval skin appendages by intraepithelial neoplasia.

In the treatment of vulval intraepithelial neoplasia (VIN) by laser, the extent to which the skin appendages are involved is an important factor in determining the depth of tissue to be destroyed. An investigation of 50 patients with VIN revealed that in 28 the skin appendages were involved and the maximum depth of involvement reached 4.6 mm from the surface of the epidermis. It is suggested that eradication of tissues to a depth of 5.0 mm would eliminate all atypical epithelium in the skin appendages.

Electrophoresis and immuno electrophoresis in leprosy.

50 cases of leprosy belonging to various subgroups i.e. 10 tuberculoid, 25 lepromatous leprosy, 10 lepra reaction, 5 dimorphic leprosy, and 25 cases or normal individuals were subjected to agar gel electrophoresis. The slides were scanned by densitometry. It showed profound departure from normal in various fractions of electrophoretic patterns. All of them showed rise of gamma globulin. Albumin was markedly decreased in lepromotous leprosy and lepra reaction. In dimorphic leprosy Alfa-1 was decreased. Qualitative immuno electrophoresis was done by using antihuman serum raised in the laboratory, by immunising rabbits. It revealed changes in IgM and IgG arcs. Results are discussed and tried to explain on immunological derangement.

Persistence of Australia antigen in leprosy--a frustrating puzzle in immunology.

This paper deals with the relationship of Australia antigen (Au) to various sub-groups of leprosy, in a total of 200 cases. Au was found to be present up to 4% in lepromatous leprosy, 2% in tuberculoid leprosy and lepra reaction, and 3% of antibody in lepromatous leprosy. Presence of antibody denotes past antigenemia. The quantitation of the antigen is done using the new technique of Electro-immuno-diffusion (EID) of Laurell, synonymous with rocket technique. The persistence of the antigen is explained in the light of deranged immunological mechanisms.