Malignant and pre-malignant oesophageal pathology in a South African teaching hospital.

BACKGROUND: South Africa (SA) has one of the highest global incidences of squamous cell carcinoma of the oesophagus (SCC). A decreasing incidence of oesophageal SCC in SA has been suggested. The study aimed to assess whether the incidence of these malignant histopathological subtypes has changed in this setting. METHOD: A retrospective review of histopathological reports on pre-malignant and malignant oesophageal lesions over three time periods (TP), namely: 2003-4 (TP1), 2008-9 (TP2) and 2013-14 (TP3) was carried out at Inkosi Albert Luthuli Central Hospital, Durban, South Africa. RESULTS: A total of 1341 specimen reports were retrieved. TP1-3 consisted of 514 (39.3%), 320 (24.5%) and 474 (36.2%) patients respectively. Six hundred and forty-nine patients were male (48.3%), 642 were female (47.8%) and 50 were not specified. i.e. a sex ratio of 1.01:1. The mean age was 60.8 (± 11.8). There were 1197 Black patients (91.5%), 66 Asian (5.1%), 25 White (1.9%), 9 mixed ancestry (0.7%), and 11 of unknown race (0.8%). SCC was the most common cancer 1098 (89.1%) followed by adenocarcinoma (AC) 69 (5.6%). The ratio of SCC to AC remained fairly consistent over the total time period. Seventy-four oesophageal resections were performed with a yearly average resection rate of only 5.6%. CONCLUSION: SCC is still the most prevalent oesophageal cancer (OC) without an increase in the ratio of AC to SCC. The diagnosis of squamous cell dysplasia is concordant with previously cited rates. Barrett's oesophagitis remains uncommon. Resection rates for OC are low but similar to other South African referring centers.

Malignant and pre-malignant oesophageal pathology in a South African teaching hospital

Background: South Africa (SA) has one of the highest global incidences of squamous cell carcinoma of the oesophagus (SCC). A decreasing incidence of oesophageal SCC in SA has been suggested. The study aimed to assess whether the incidence of these malignant histopathological subtypes has changed in this setting. Methods: A retrospective review of histopathological reports on pre-malignant and malignant oesophageal lesions over three time periods (TP), namely: 2003­4 (TP1), 2008­9 (TP2) and 2013­14 (TP3) was carried out at Inkosi Albert Luthuli Central Hospital, Durban, South Africa.Results: A total of 1341 specimen reports were retrieved. TP1-3 consisted of 514 (39.3%), 320 (24.5%) and 474 (36.2%) patients respectively. Six hundred and forty-nine patients were male (48.3%), 642 were female (47.8%) and 50 were not specified. i.e. a sex ratio of 1.01:1. The mean age was 60.8 (± 11.8). There were 1197 Black patients (91.5%), 66 Asian (5.1%), 25 White (1.9%), 9 mixed ancestry (0.7%), and 11 of unknown race (0.8%). SCC was the most common cancer 1098 (89.1%) followed by adenocarcinoma (AC) 69 (5.6%). The ratio of SCC to AC remained fairly consistent over the total time period. Seventy-four oesophageal resections were performed with a yearly average resection rate of only 5.6%.Conclusions: SCC is still the most prevalent oesophageal cancer (OC) without an increase in the ratio of AC to SCC. The diagnosis of squamous cell dysplasia is concordant with previously cited rates. Barrett's oesophagitis remains uncommon. Resection rates for OC are low but similar to other South African referring centers

Endovascular Therapy for Large Vessel Vasculopathy in HIV-infected Patients.

OBJECTIVES: To evaluate outcomes after endovascular treatment of patients with aneurysmal or occlusive vasculopathy in HIV-infected patients. METHODS: Retrospective analysis of a prospective database of treatment outcomes in patients with HIV related vasculopathies between April 2005 and September 2015. RESULTS: Sixty HIV patients presented with post-traumatic pseudoaneurysm formation (n = 7), aneurysmal disease (n = 24) or occlusive disease (n = 29 (48%)). The majority were male (42/60 (70%)), with a mean age of 43.9 years (SD ± 12.6). All seven patients with a post-traumatic pseudoaneurysm were treated by insertion of a covered stent (n = 6) or coiling (n = 1). All were successfully treated at 30 days, but only one patient returned for late surveillance. 23/24 patients who underwent insertion of a stent graft/covered stent for aneurysmal disease returned for 30 day review (one asymptomatic stent graft occlusion). Only 11 patients attended for late surveillance; 9/11 were asymptomatic with patent stent grafts. Late stent occlusion occurred in two (no further action (n = 1), major limb amputation (n = 1). In the 29 patients who underwent endovascular treatment for occlusive disease, 9 (31%) had immediate treatment failure (including 8 amputations (28%)). Of the sixteen who returned for serial review, 8 (50%) suffered further complications including 4 amputations. Overall, 12/29 treated patients (41%) ultimately underwent amputation. CONCLUSIONS: In the immediate short term, an 'endovascular first' strategy was associated with good outcomes in HIV patients with aneurysmal disease. By contrast, outcomes were poor in HIV patients with occlusive disease. Whether this relates to the underlying natural history of HIV occlusive vasculopathies remains unclear. One major problem in trying to formulate meaningful management strategies is a generalised reluctance for HIV patients to return for surveillance.

Bilateral Wilms' tumour in a developing country: a descriptive study.

PURPOSE: To present our experience of 20 children with bilateral Wilms' tumour seen in a resource-challenged environment over a 10-year period. METHOD: All patients with a diagnosis of bilateral synchronous Wilms' tumour were identified and recruited. RESULTS: Study patients represented 11 % of a cohort of 177 new patients with Wilms' tumour seen over the same period. Three patients had a syndromic predisposition to Wilms' tumour. Metastatic disease was seen at presentation in four patients (20 %) and three children presented with unilateral tumour rupture. One patient presented with paraplegia and one with obstruction of the duodenum. All children received neoadjuvant chemotherapy. One HIV-infected child died of IRIS after neoadjuvant treatment, but before surgery. One child died of progressive disease after unilateral nephrectomy. Nephron-sparing surgery was performed in 22 kidneys and 15 kidneys were removed in toto. Following enucleation of tumours, three children had positive margins. Discordant histopathology was seen in 53 % of patients. Overall survival at 2 years is 85 %. CONCLUSION: Despite significant co-morbidity and advanced disease, bilateral Wilms' tumour is a treatable disease in a resource-constrained environment.

Oral cavity squamous cell carcinoma metastasis to the submandibular gland.

INTRODUCTION: This study aimed to evaluate the incidence of submandibular gland metastases in cases of oral cavity squamous cell carcinoma. METHODS: Sixty-nine patients treated between 1 January 2004 and 30 June 2009 were retrospectively reviewed. Patients were treated with wide local excision of the primary tumour, plus simultaneous neck dissection and reconstruction if required. RESULTS: Of 69 submandibular glands from 46 men and 23 women (mean age, 58 years), 43 (62.3 per cent) had advanced, tumour stage three or four lesions. Histopathological reappraisal of all submandibular glands demonstrated an absence of metastatic spread. Only 2/69 (2.9 per cent) submandibular glands demonstrated ipsilateral contiguous tumour involvement. CONCLUSION: This study demonstrated an absence of metastasis to the submandibular gland from oral cavity squamous cell carcinoma. Patients with early stage oral cavity squamous cell carcinoma and with a pre-operative node stage zero neck may be candidates for preservation of the submandibular gland during neck dissection.

Intestinal inflammatory myofibroblastic tumour.

BACKGROUND: Inflammatory myofibroblastic tumours (IMFTs) are rare tumours characterised by nosologic, histogenetic and aetiopathogenetic controversy and variable clinicopathological features. We report our experience with intestinal-IMFTs (I-IMFTs) that have been reported mainly as single case reports to date. METHODS: Five patients with I-IMFTs, identified between 2005 and 2008, formed the study cohort. The clinicopathological features were obtained from departmental and hospital records. RESULTS: The median patient age was 13 years. While 4 patients presented with symptoms and signs of intestinal obstruction, one IMFT was an incidental finding at laparotomy for trauma. Three I-IMFTs were located in the small bowel and 2 in the colon. Complete resection with end-to-end anastomoses was performed. The gross morphology included 1 polypoid myxoid tumour that served as a lead point for an intussusception, 3 multinodular whorled masses and 1 firm circumferential, infiltrative tumour. Microscopically, all tumours had typical features of IMFT with variable expression of ALK-1, a low proliferation index and tumour-free resection margins. All patients had an uneventful recovery. One patient was lost to further follow-up. Four patients were well, without local recurrence or metastases at 6 months to 3 years. CONCLUSIONS: Surgery with tumour-free resection margins is the gold standard of care of adult and paediatric I-IMFTs. Heightened recognition of I-IMFT, albeit rare, as a cause of intestinal obstruction, including intussusception, is necessary for pre-operative suspicion of I-IMFT.

Spinal cord compression in children with Wilms' tumour.

PURPOSE: To document the clinical, imaging and histopathological features of five children with paraplegia due to Wilms' tumour (WT), highlighting therapeutic options and patient outcome in a developing country. METHODS: Patients with WT and paraplegia seen at the Department of Paediatric Surgery since 1984 form the study cohort. Patient demographics, duration of neurological symptoms, stage of primary tumour, therapeutic intervention and outcome were recorded. Histology of the primary tumours and paraspinal or epidural biopsies were reviewed. RESULTS: Five patients with WT and paraplegia were identified. Imaging showed epidural masses with paraspinal disease, cord displacement and compression. Four patients have died. Of the two patients with neurological recovery, one relapsed 4 months later. Histology revealed triphasic WT with one case showing anaplasia. Paraspinal or epidural biopsies confirmed WT with post-treatment changes. Three biopsies showed lymphovascular, perineurial and intraneural tumour invasion and one showed epidural venous invasion. CONCLUSION: Although rare, WT-associated spinal disease may cause permanent neurological deficit, adding considerably to the burden of disease. In developing countries where patients present late, the prognosis is poor, however surgery may provide immediate relief of compression symptoms and biopsy material. The treatment of choice will depend on the facilities available and the clinical circumstances.

The gonads of 111 South African patients with ovotesticular disorder of sex differentiation.

PURPOSE: The aims of this study were to describe the gonadal tissue found in the Southern African true hermaphrodite and establish if there was a correlation between the clinical and histopathologic findings and if these findings were similar to patients with this condition elsewhere. MATERIALS AND METHODS: A retrospective study at the University of KwaZulu-Natal, Durban, South Africa, looked at all patients diagnosed with true hermaphroditism seen between 1984 and 2006. For this 23-year period, 111 consecutive true hermaphrodite patients were diagnosed on clinical findings, internal genital assessments, and the histologic examination of 217 gonadal biopsy specimens. All gonadal tissue taken from these patients was sent for histopathologic evaluation. The results were correlated to the clinical and internal genital evaluations of the patients. RESULTS: Five patients only had a single gonad. Analysis of the gonadal biopsy specimens showed that there were 118 (54%) ovotestes together with 59 ovaries and 40 testes. The ovotestes were divisible on gross appearance into 11% bipolar and 89% mixed types. Histologically, the mixed-type ovotestes have an outer mantle consisting of ovarian tissue, which encapsulated an inner core of 2 distinct types. The first is an admixed ovotestis (constituting 44% of the mixed ovotestes), the central core consisted of gonadal stroma, with scattered foci of separate ovarian and testicular tissue. The second type was the compartmentalized ovotestis (constituting 56% of the mixed ovotestes); here, the outer mantle was thickened in the upper pole and encapsulated a large core of testicular tissue in the lower pole of the gonad. The bipolar ovotestis had a strictly polar distribution of ovarian and testicular tissue, which had an irregularly interdigitating junction between the 2 types of tissue. Statistical analysis showed that no correlation could be found between the type of gonadal tissue and any of the clinical or genital features. CONCLUSION: Three distinct ovotesticular types are identified in the Southern African true hermaphrodite, which have not been described previously. The structure of these gonads has bearing on the type of biopsy done and the subsequent management of the ovotestes.

Drug-induced cutaneous pathology.

Drug-induced cutaneous rashes, whether confined to the skin or part of a systemic disease, are characterised by a spectrum of inflammatory disease patterns that include perivascular dermatitis, nodular and diffuse dermatitis, vesiculobullous lesions, pustular eruptions, sclerodermoid reactions, vasculitis, folliculitis/perifolliculitis and panniculitis. While a single drug can elicit a range of reaction patterns, no reaction pattern is specific for a particular drug. Although the temporal link between initiation of drug therapy and the onset of the drug rash is critical to the diagnosis, drug reactions may also occur during the course of chronic drug ingestion. Clues to the drug-induced nature of the cutaneous eruption include the presence of overlapping histological reaction patterns and incongruent clinical and histopathological features. While eosinophils are an important tell-tale sign of a drug-induced reaction, they may also be conspicuous in skin rashes devoid of a drug association. Furthermore, eosinophils may be sparse or absent in some drug exanthems. Heightened awareness of the mimicry of a wide spectrum of cutaneous pathology by an ever-increasing range of therapeutic agents is pivotal to the diagnosis of drug-induced skin pathology.

Lymphoepithelial lesions of the parotid gland in the HIV era--a South African experience.

OBJECTIVE: Historically, lymphoepithelial lesions (LELs) have been a peripheral indication for surgery on the parotid gland, accounting for less than 1% of all parotidectomies undertaken. In the HIV era the profile of parotidectomy has changed, with LEL becoming a common indication for parotidectomy, prompting a review of our current experience. DESIGN: A retrospective study was undertaken between January 1998 and December 2005. Setting. Surgical services at King Edward VIII Hospital, Durban. SUBJECTS: All patients presenting with parotidomegaly. RESULTS: One hundred and sixty-two patients with parotidomegaly were evaluated; 53 (32.7%) had LEL lesions. A total of 151 parotidectomies (including 42 parotidectomies undertaken for LEL lesions) were performed in 147 patients. Radiotherapy was offered to 11 patients with LEL lesions. Complications following parotidectomy for LEL included facial nerve palsy (N=3), seroma formation (N=5) and facial artery false aneurysm (N=1). Three patients required re-parotidectomy for recurrent LEL parotidomegaly. In the patients who underwent radiotherapy, there was a satisfactory outcome; 2 patients who received 4 Gy radiation per sitting developed mild skin discolouration of the treated area. CONCLUSION: Although surgical management of LEL parotidomegaly may provide a satisfactory result, this approach is technically challenging and is associated with complications and recurrences. For these reasons, the non-operative approach employing radiotherapy should be considered.

A cross-sectional study of HIV-seropositive patients with varying degrees of proteinuria in South Africa.

Human immunodeficiency virus (HIV)-associated nephropathy (HIVAN) is the most common finding on renal biopsy in HIV-infected black patients and is also the commonest cause of end-stage renal disease in these patients. Early detection of HIVAN may be beneficial in evaluating early treatment. This study examined the pattern of renal diseases in HIV-infected South Africans and also attempted to diagnose HIVAN at an early stage. In this single-center cross-sectional study, 615 HIV-infected patients were screened for proteinuria. Thirty patients with varying degrees of proteinuria underwent renal biopsy. Patients with diabetes mellitus, uncontrolled hypertension, known causes of chronic kidney disease, and serum creatinine above 250 mumol/l were excluded. Patients in this study were not on antiretroviral therapy. HIVAN was found in 25 (83%) patients. Six of them (24%) had microalbuminuria. Altogether, seven patients with persistent microalbuminuria were biopsied and six (86%) showed HIVAN. Other biopsy findings included membranoproliferative nephropathy in two (7%) and interstitial nephritis in three (10%). Four patients with HIVAN had associated membranous nephropathy. HIVAN is the commonest biopsy finding among our study patients with HIV infection who present with varying degrees of proteinuria. Microalbuminuria is a manifestation of HIVAN in our study patients. Therefore, microalbuminuria may be an early marker of HIVAN, and screening for its presence may be beneficial. Renal biopsy may be considered in seropositive patients who present with persistent microalbuminuria, especially with low CD4 counts irrespective of good renal function. This will allow diagnosis and treatment of HIVAN at an early stage and may prevent further disease progression.

Isolated splenic tuberculosis.

Tuberculosis isolated to the spleen is a rare clinical entity particularly in the non-HIV-positive patient population. In the four patients described, two presented with thrombocytopenia; in two patients the condition was diagnosed serendipitously at laparotomy undertaken for abdominal trauma.

Isolated amoebic appendicitis.

Amoebiasis, a disease of worldwide distribution, is endemic in tropical countries with suboptimal sanitation facilities. Isolated amoebic appendicitis (IAA) is regarded as a rare manifestation of the disease globally. Because there are no defined clinical features that distinguish IAA from bacterial appendicitis, diagnosis is usually dependent on histopathological examination. A 9-year retrospective study was undertaken to investigate the clinicopathological aspects of IAA. The main complaints were fever and abdominal pain. None of the patients had dysentery. The pre-operative clinical diagnosis was acute appendicitis and acute abdomen in 13 and 8 patients, respectively. In all cases the intra-operative diagnosis was acute appendicitis. Gross pathological appraisal revealed peritonitis and perforation in 19 and 17 cases, respectively. Histopathological examination of these appendices demonstrated appendiceal ulceration, transmural mixed inflammation, haematophagous amoebic trophozoites and necrosis in all cases. Vascular pathology comprised venous and capillary luminal plugging (11 cases), necrotising small vessel vasculitis (11 cases), thrombophlebitis of medium sized veins (9 cases) and arteritis with associated thrombosis (1 case). Organising fibrinopurulent peritonitis was present in 19 cases. Two appendices that appeared normal macroscopically demonstrated ulceration and inflammation that were confined to the mucosa and submucosa. All of 18 patients who were treated with metronidazole survived without further surgery, while three patients who were untreated succumbed to the disease. Appendicectomy, accurate histopathological appraisal thereof and optimal, timely management of IAA were critical to the favourable outcome in the present study.

Pemphigus in South Africa.

BACKGROUND: Pemphigus is an autoimmune disease characterized by intraepidermal blistering. We describe the demography, prevalence, clinical features, response to treatment, and human leukocyte antigen (HLA) characteristics of pemphigus in Kwa-Zulu Natal, South Africa. METHODS: All patients with pemphigus were prospectively recruited over 12 years from January 1987 to December 1999. The demography, clinical features, histology, and immunofluorescence (IF) were recorded. In a subset of patients, HLA tests were performed. RESULTS: One hundred and twelve patients had pemphigus. Pemphigus foliaceus (PF) was the commonest variant seen (62 patients) and 80% of these patients were black. The mean age was 43 years (12-93 years) and the male to female ratio was 1 : 1.4. Fifty patients had pemphigus vulgaris (PV), of whom 82% were Indian. The mean age of presentation of PV was 48 years (21-82 years). The male to female ratio was 1 : 1.7. There was no mucosal involvement in PF. PV patients had painful oral lesions. The mortality rate was 14% in the total sample (six in PV and two in PF). HLA-B8 was positive in 41% of patients with PF (P < 0.001). CONCLUSIONS: PF occurs more commonly in black people, while most cases in Indians present with the PV subtype. Pemphigus patients present with severe and extensive disease, and PV patients share features in common with patients from their land of origin (India), suggesting a genetic link.

Aggressive CD34-positive fibrous scalp lesion of childhood: extrapulmonary solitary fibrous tumor.

Although solitary fibrous tumor (SFT) was originally described as a pleural tumor, an increasing number of extrapleural sites of SFTs have been documented. This has been attributed not only to the heightened awareness of the spectrum of histopathological features that characterizes SFTs but also to the recognition of the role of CD34 immunostaining in soft tissue tumors in general, and in SFTs in particular. Despite the large number of documented extrapleural SFTs in adults, cranial SFTs are rare, having been documented in the meninges, scalp, and infratemporal fossa. Extrapleural SFTs are, to date, an unrecognized entity in children. We document an aggressive fibrous scalp lesion in a 30-month-old female child that demonstrated features common to benign cranial fasciitis and SFT. However, based on bright, diffuse CD34 antigen immunopositivity, a diagnosis of SFT was made. The need to include the CD34 antigen stain in a panel of immunohistochemical markers used to assess spindle cell lesions of childhood is emphasized.

Transepithelial elimination of late cutaneous vulvar schistosomiasis.

Late cutaneous vulvar schistosomiasis (LCVS), which represents the cutaneous response to the deposition of schistosomal ova, is characterized by a range of clinical manifestations. Histopathological descriptions of LCVS have highlighted the hyperplastic epithelial reaction, and a few reports have alluded to the presence of intraepidermal bilharzial ova. Although transepithelial elimination (TEE), a well-known phenomenon whereby the skin rids itself of foreign, potentially dangerous substances, has been documented in a range of infectious processes, it has not been recognized as a distinct process in LCVS. This study not only documents TEE in 23 biopsies of LCVS but also correlates the role of the histopathological inflammatory reaction pattern, density of ova, and pseudoepitheliomatous hyperplasia in the pathogenesis of TEE. The importance of TEE as an additional, hitherto unrecognized mechanism of release and spread of schistosomal ova to the exterior is also highlighted.