Pratistadevi K. Ramdial

Disseminated cutaneous histoplasmosis in patients infected with human immunodeficiency virus

Background: In the pre‐AIDS era disseminated histoplasmosis was rare and the cutaneous manifestations thereof were reported infrequently. A range of unusual clinical manifestations of disseminated cutaneous histoplasmosis (DCH) in AIDS patients has been documented, but the cutaneous histopathological descriptions are short and incomplete. In addition, the histopathological spectrum of AIDS‐associated DCH is poorly recognized.

Pemphigus in South Africa

Abstract

An Unusual Cause of Proptosis: Orbital Solitary Fibrous Tumor: Case Report

Although solitary fibrous tumors are well-recognized tumors, they have only recently been described in the orbit. Although histopathological awareness of the lesion has been heightened recently because of the discovery of CD-34 immunoreactivity in solitary fibrous tumors, including orbital lesions, it remains unrecognized clinically as a cause of unilateral proptosis. Clinical awareness of the lesion is important, because benign and malignant forms of the tumor occur. The malignant forms pursue an aggressive course manifested by local invasion, recurrent growth, or metastases. The benign lesions are histologically banal and are cured by surgical excision. This case report describes unilateral proptosis caused by an orbital solitary fibrous tumor, which occurred in a 43-year-old woman. The tumor had a benign histomorphology and clinical course, with surgical excision being curative. In reviewing the literature on orbital solitary fibrous tumors, clinical awareness of the tumor as a cause of unilateral proptosis is emphasized.

bcl-2 protein expression in aggressive and non-aggressive basal cell carcinomas.

bcl‐2, the well known anti‐apoptotic gene, cloned more than a decade ago, promotes cell viability without promoting cell proliferation. With few exceptions, high bcl‐2 protein expression is associated with a favourable outcome in epithelial tumours. bcl‐2 immunoreactivity in basal cell carcinomas (BCCs) is contradictory, with 67–100% immunopositivity being reported. Although BCCs are traditionally regarded as low‐grade, indolent tumours, aggressive BCCs (A‐BCCs) are mutilative, locally destructive tumours that often recur. bcl‐2 protein expression as a predictor of BCC aggressiveness is poorly documented in the English‐language literature. The bcl‐2 protein immunoprofile of 50 clinically non‐aggressive (NA‐BCCs) and 25 clinically A‐BCCs was investigated. Of the latter, 17 manifested with one, two or three recurrences. bcl‐2 protein expression in each of the recurrences was also evaluated. bcl‐2 expression was scored as follows: 0–5% positive cells=negative, 6–25%=1+, 26–50%=2+, 51–75%=3+, >75%=4+. “High” labeling encompassed 3+ or 4+ labeling while “low” labeling referred to 1+ or 2+ labeling. Although bcl‐2 positivity was noted in all BCCs, low bcl‐2 labeling was a statistically significant feature of A‐BCCs (p < 0.01). High bcl‐2 labeling of NA‐BCCs was a reflection of the bcl‐2 labeling of the dominant constituent nodular or superficial subtypes. Micronodular BCCs revealed 2+ or 3+ labeling. Initial and recurrent A‐BCCs with a pure or predominantly infiltrative component, demonstrated 1+ or 2+ bcl‐2 labeling. The differential bcl‐2 expression in the various clinicopathological subtypes of BCCs suggests that, despite the common derivation of these tumours from a primitive basaloid stem cell and a limited potential for metastasis, they form a heterogeneous group of tumours that differ markedly in histologic and biological behaviour. While the superficial and nodular BCCs are indolent slow‐growing tumours with high bcl‐2 labeling, the aggressive BCCs are infiltrative, desmoplastic tumours with low bcl‐2 labeling. In mixed tumours, heterogeneity of labeling is a distinctive feature and is contributed to in part by the labeling trends of the different histological subtypes. The micronodular BCC shows varied bcl‐2 labeling but in combined tumours occupies a niche intermediate between the non‐aggressive nodular and superficial and the aggressive infiltrative subtypes. The initial and subsequent biopsies of recurrent, adequately excised BCCs share a pure or mixed, predominantly infiltrative, stroma‐rich histomorphology with low bcl‐2 labeling, reflecting the immunoprofile of a more aggressive growth pattern.

Erythroderma: a comparison between HIV positive and negative patients

Background Erythroderma has protean underlying causes. There have been isolated case reports suggesting an association between erythroderma and the human immunodeficiency virus (HIV).

Mucinous tubular and spindle cell carcinoma with aggressive histomorphology—a sarcomatoid variant

Mucinous tubular and spindle cell carcinoma (MTSCC) is a recently described renal epithelial tumor. The bland cytomorphology of the spindled component and low-grade behavior help in its differentiation from sarcomatoid renal carcinoma. Sarcomatoid change has been reported in most histologic variants of renal cell carcinoma apart from MTSCC. Herein we report a case of an MTSCC in a 72-year-old female patient with high-grade spindled areas resembling fibrosarcomatous and undifferentiated pleomorphic sarcoma patterns with metaplastic bone. This index case also demonstrates a high proliferation index and extensive necrosis representing the first documented case of sarcomatoid change in MTSCC.

Cryptococcal inflammatory pseudotumors.

“Inflammatory pseudotumors” (IPTs) embrace a heterogeneous spectrum of reactive, infective, and neoplastic entities, that are characterized by a clinical mass composed of a histologic proliferation of spindle cells in a background of inflammatory cells and collagen fibers. Although a spectrum of microorganisms have been identified in infective IPTs, mycobacterial infective IPTs are reported most commonly. We document 5 solitary cryptococcal IPTs, in 2 males and 3 females, aged 19 to 43 years, in the soft tissues of the anterior chest wall, thigh, and arm. All were HIV-positive and had been treated for disseminated cutaneous and/or meningeal cryptococcosis with antifungal therapy, 6 to 12 months earlier. The specimens demonstrated a storiform arrangement of plump spindle cells, in addition to spindle and polygonal cells that were arranged in a haphazard manner. Background lymphocytes, plasma cells, and fibrosis were noted, in addition to scattered giant cells and focal necrosis. On high-power examination, Cryptococcus neoformans yeasts were identified within and between vacuolated spindle and polygonal cells on routine and special stains, confirming cryptococcal IPTs. Immunophenotyping of the spindle cells confirmed a mixed histiocytic and myofibroblastic lineage, with a predominance of the former. In documenting 5, hitherto unreported, pseudotumoral spindle cell reactions to C. neoformans, we not only highlight the need for intense appraisal of all IPTs for infective agents on routine and special stains and investigations, but also postulate that a complex host-fungus interaction, coupled with an exuberant, myofibroblastic response to incomplete therapy, are the pathogenetic drive for the pseudotumoral presentation.

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.

Intracranial tuberculous subdural empyema: case report.

OBJECTIVE AND IMPORTANCE Many types of neurotuberculosis have been described; the most common intracranial forms are tuberculous meningitis and tuberculomas. We report a unique and as yet unreported form of neurotuberculosis, which is an intracranial tuberculous subdural empyema. CLINICAL PRESENTATION A 59-year-old man who had been previously treated for pulmonary tuberculosis (TB) presented at our institution with a long-standing history of headaches. General and neurological examinations revealed no abnormalities. Radiography of the chest confirmed fibrotic lung changes caused by healed pulmonary TB. A cranial computed tomographic scan revealed a hypodense extra-axial collection with mass effect as well as adjacent osteitis and scalp swelling. INTERVENTION The patient underwent craniectomy of the osteitic bone and drainage of 50 ml of fluid pus located subdurally. Microscopic examination of the bone and pus revealed tuberculous granulation tissue with numerous acid-fast bacilli identified using Ziehl-Neelsen stain. Mycobacterium TB bacillus was cultured from the pus at 42 days. The patient required two further operative procedures as well as a protracted course of anti-TB therapy. CONCLUSION The patient eventually achieved a good recovery. We recommend surgical drainage of tuberculous subdural empyema to relieve mass effect and to obtain microbiological confirmation. Furthermore, surgical treatment should be combined with an 18-month course of anti-TB chemotherapy, during which period patient compliance should be closely monitored.

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.