Apophysomyces variabilis, an emerging and worrisome cause of primary cutaneous necrotizing infections in India.

In India, Apophysomyces species complex is the second common agent of mucormycosis mainly affecting immunocompetent hosts unlike other Mucorales. It is frequently involved in causing necrotizing cutaneous infections. The present two-year study was planned to investigate the causative role of Apophysomyces spp. in cutaneous necrotizing infections. The tissue samples were processed using standard techniques and in five out of 65 patients, Apophysomyces variabilis was the etiological agent. Diabetes mellitus and trauma were common risk factors and despite appropriate treatment only one patient could be survived. Apophysomyces variabilis is emerging as agent of necrotizing infections which invariably result in poor patient outcomes.

Limb Salvage Using Liquid Nitrogen-Treated Tumour-Bearing Autograft: A Single Institutional Experience of 10 Patients.

BACKGROUND: Many reconstruction methods have evolved to offer limb salvage surgery (LSS) to patients with musculoskeletal sarcomas. It can be achieved using endoprosthesis or biological reconstruction methods like allograft or autograft or a combination of both. In carefully selected patients, resected bone can be recycled and reimplanted after sterilisation using methods like irradiation, autoclaving, pasteurisation or liquid nitrogen. METHODS: From 2010 to 2016, 10 patients with primary musculoskeletal sarcoma underwent limb salvage surgery (LSS) by wide resection of the tumour and reconstruction using recycled autograft treated with liquid nitrogen. Intercalary resection was carried out in six patients and intra-articular in four. The resected bone was dipped in liquid nitrogen for 25 min, thawed at room temperature for 15 min followed by dipping in vancomycin-mixed saline for 10 min. The recycled bone was re-implanted into its original site and stabilised with internal fixation. RESULTS: At a mean follow-up period of 39.6 months (range 6-97 months), all patients had a good function (mean functional score of 80%) with no evidence of local recurrence in the re-implanted bone or otherwise. Union was achieved at 15 of the 16 osteotomy sites with a mean union time of 5.2 months (range 4-7 months) without any additional surgical interventions. In none of the patient, augmentation with vascularised/non-vascularised fibula was done. No complication like fracture of the autograft, implant failure or deep/superficial infection was reported in any patient. CONCLUSION: Recycled tumour-bearing autograft after treatment with liquid nitrogen is an anatomical, cost-effective, relatively simpler and reliable technique for reconstruction of bone defect after resection in selective primary musculoskeletal sarcoma patients.

Evaluating the Unusual Histological Aspects of Granuloma Annulare: A Study of 30 Cases.

BACKGROUND: Granuloma annulare (GA) is an uncommon dermatologic disorder that presents as annular, skin-colored to erythematous plaques. Histopathologically, it is characterized by palisaded histiocytic granulomas. A definitive diagnosis of GA is based on clinicopathologic correlation. OBJECTIVE: The aim of this study was to study the histomorphologic spectrum of GA. MATERIALS AND METHODS: A total of 30 cases reported as GA over 6 years (2012-2017) were retrieved. The detailed clinical profile and histomorphologic findings on the skin biospies were reviewed. RESULTS: Majority of the cases (40%) presented in the 6th decade of life with a mean age of 48.3 ± 16.5 years and with a female predominance (77%). The lesions were localized in 22 cases (73%). Asymptomatic to erythematous, annular plaques was the most frequent presentation (60%). GA was not suspected clinically in two cases. Histopathologically, interstitial pattern of infiltrate was most common (44%), whereas granuloma formation and palisaded histiocytes were seen in 4 (13%) and 3 cases (10%), respectively. A mixed pattern was observed in 10 (33%) cases. Collagen degeneration was universal finding (100%) and presence of dermal mucin was noted in 24 cases (80%), both of which were important clues to the diagnosis of GA. Additional features such as presence of plasma cells, eosinophils, and vasculitis were noted in 10 (33%), 6 (20%), and 6 (20%) cases, respectively. CONCLUSION: The diagnosis of GA may be challenging owing to its diverse morphology. Acquaintance with the varied histomorphology of GA is of utmost importance to render a correct diagnosis and understand the pathogenesis.

Mucormycosis: Battle with the Deadly Enemy over a Five-Year Period in India.

Mucormycosis is an emerging opportunistic fungal infection. Increasing immunocompromization, widespread use of antibacterial and antifungal agents (such as voriconazole prophylaxis), carcinomas, transplantation and lifestyle diseases such as diabetes are the main contributors to this situation. The predominant clinical manifestations of mucormycosis vary from host to host, with rhino-orbital-cerebral, pulmonary, cutaneous, and gastrointestinal infections being the most common. In India, the prevalence of mucormycosis is approximately 0.14 cases/1000 population, which is about 70 times the worldwide-estimated rate for mucormycosis. The present study was undertaken over a period of five years (January 2009-December 2014) to determine the prevalence of mucormycosis. The samples suspected of mucormycosis were examined by direct KOH wet mount and cultured on Sabouraud's dextrose agar without actidione and on blood agar as per standard mycological techniques. Histopathological correlation was done for most of the cases. Antifungal susceptibility testing was performed by the EUCAST reference method. We identified a total of 82 cases of mucormycosis out of a total of 6365 samples received for mycological culture and examination during the said time period. Out of these, 56 were male patients and 27 were females. Most common presentation was rhino-orbito-cerebral (37), followed by cutaneous (25), pulmonary (14), oral cavity involvement (4) and gastrointestinal (2). The most common risk factors were diabetes and intramuscular injections. The fungi isolated were Rhizopus arrhizus (17), Apophysomyces variabilis (12), R. microsporus (9), Lichtheimia ramosa (8), Saksenaea erythrospora (5), Syncephalastrum racemosus (4), R. homothallicus (2), Rhizomucor pusillus (1), Mucor irregularis (1) and A. elegans (1). The mainstay of the treatment was amphotericin B, along with extensive surgical debridement whenever feasible. Most of the patients (50) recovered, but 25 died. The rest of the patients left against medical advice. "Nip in the Bud" should be the mantra for clinicians/surgeons for a favorable prognosis. Early diagnosis, prompt institution of appropriate antifungal therapy, surgical debridement whenever necessary, knowledge of risk factors and their timely reversal is the key for management.

Intraoral and Oropharyngeal Lesions: Role of Fine Needle Aspiration Cytology in the Diagnosis.

The oral cavity is affected by a wide range of pathologic lesions, for which a morphologic diagnosis is required for proper management. Fine needle aspiration (FNA) is being increasingly used for preliminary diagnoses of such lesions. This is retrospective analysis of intraoral and oropharyngeal lesions diagnosed with FNAC over a period of 7 years. Out of total 55 cases, a definite diagnosis could be made on cytology in 50 cases (90.9 %). These 50 cases were further included in the study. Thirty cases were reported as non-neoplastic and 20 as neoplastic (11 benign and nine malignant). The diagnoses were made taking into account the background material (blood, mucin) and the predominant cells present (neutrophils, lymphoid cells, macrophages, hemosiderin laden macrophages, squamous cells, basaloid cells, spindle cells, giant cells). Histopathological diagnosis was available in 17 cases and corresponded with FNA diagnosis in 16 cases (94.12 %). No significant complications were seen in patients undergoing these FNAs. It can be concluded that FNA is a simple and rapid diagnostic test that can be useful for preliminary assessment of oral and oropharyngeal lesions.

Clinico-pathological study of glomerular diseases in patients with significant proteinuria in North India.

Proteinuria is a common manifestation of renal disease. The present study was carried out to analyze the clinic-pathological correlation, assess the value of histopathology and immunofluorescence (IF) as well as note the spectrum of renal diseases in patients with significant proteinuria. Fifty consecutive patients having proteinuria >1 g/24 h underwent ultrasound-guided percutaneous renal biopsy. Clinical information was correlated with the pathological findings and the results were analyzed. The patients were in the age range of 12-79 years. Males (60%) outnumbered females (40%) in all the disease categories except lupus nephritis and IgA nephropathy. The most common clinical presentation was the nephrotic syndrome, seen in 31 cases (62%). Primary glomerular diseases (72%) were more common than secondary glomerular diseases (24%) and tubulointerstitial diseases (4%). Overall, the most common pathological diagnosis was focal and segmental glomerulosclerosis (FSGS) (20%), followed by membranous glomerulonephritis (MGN) (18%). In young patients (age <20 years), minimal change disease (36.4%) was the most common diagnosis while in adults it was MGN (23.5%) and in elderly patients (age >60 years) it was FSGS (60%). IF modified the diagnosis in 12% of the cases. The concordance between clinical diagnosis and pathological diagnosis was 66%. The difference between clinical diagnosis and final diagnosis was statistically significant. Our study further reinforces the knowledge that renal biopsy helps in accurate diagnosis and, thus, helps in appropriate management of the patients. IF provides additional information that can make the morphologic diagnosis considerably more precise.

Paraneoplastic erythrocytosis in a malignant ovarian steroid cell tumour.

Neoplastic polycythemia is a condition in which high hematocrit levels result from a neoplastic lesion in the body. It is a rare condition and occur because of excess erythropoietin or androgen production by the neoplastic cells. Steroid cell tumours of the ovary though notorious for producing endocrine changes in the body have rarely been associated with polycythemia. We present the case of a post menopausal lady who developed polycythemia and features of virilisation. Further investigations revealed that she had steroid cell tumour of the left ovary. Her signs and symptoms resolved after surgical removal of the tumour.

Fine needle aspiration cytology: a useful technique for diagnosis of invasive fungal rhinosinusitis.

Mycotic infections are on the rise globally. Patients with invasive fungal infection of the paranasal sinuses often present with destructive mass lesions and mimic malignancy clinically and radiologically. To assess the utility of Fine needle aspiration cytology for early diagnosis of invasive fungal rhinosinusitis. Fine needle aspiration cytology was performed from the maxillary/ethmoid sinus in patients with a destructive mass lesion in the maxilla. Differential diagnoses were malignancy and fungal rhinosinusitis. In eight cases fungi were detected on initial examination whereas in a single case that was initially reported as giant cell lesion, hyphae could be identified within giant cells, on review. Smears showed inflammatory cells with variable numbers of eosinophils with neutrophils and histiocytes. Foreign body giant cells were seen in all cases. The fungi conformed to morphology of aspergillus in seven cases (77.78%); in two cases (22.22%), typing could not be done. Periodic acid Schiff and Grocott stains highlighted the fungi in all the cases. Fine needle aspiration is a simple technique that can be useful for diagnosis of fungal rhinosinusitis and to exclude malignancy. Search for fungus may be more aggressive in smears with many foreign body giant cells and inflammatory cells and in cases with a high clinical suspicion. Differentiation between aspergillus and mucor can be made with help of special stains. Aspergillus is the commonest agent isolated. Preoperative cytological diagnosis obviates the need for biopsy, saves time and helps to plan proper treatment.

Kimura's disease: clinicopathological study of eight cases.

BACKGROUND: Kimura's disease is a rare, localised, chronic inflammatory disease. This benign disease involves subcutaneous tissues, the major salivary gland, and lymph nodes primarily in the head and neck area. METHOD: Clinical details and stained slides of all cases reported as Kimura's disease over a 10-year period were reviewed. RESULTS: There were eight cases of Kimura's disease. The mean age of patients was 22.8 years. One case showed associated nephrotic syndrome and two cases were associated with peripheral blood eosinophilia. All cases showed the typical histopathological features of Kimura's disease. CONCLUSION: Kimura's disease was first reported in China in 1937. The cause of Kimura's disease is unknown and many theories have been proposed. The eight cases reported here illustrate some of the variations in the mode of presentation and in the histological features of Kimura's disease. Kimura's disease should be considered in the differential diagnosis of patients who present with head and neck swellings and lymphadenopathy, and investigated accordingly.

Cytologic diagnosis of intravascular papillary endothelial hyperplasia: a report of two cases and review of cytologic literature.

BACKGROUND: Intravascular papillary endothelial hyperplasia (IPEH), previously known as 'Masson's hemangioma', is a reactive endothelial proliferation that occurs most commonly in the vessels of the head, neck, and extremities. The cytologic findings of the lesion are varied and depend on the age of the lesion. CASES: Case 1 is a 61-year-old man who presented with a swelling on the medial aspect of the forearm. The clinical diagnosis was lipoma. Cytologic smears showed spindle cells tagging onto a rich capillary network and smaller round cells arranged around hyaline cores. The cytologic diagnosis was benign vascular tumor. On histolopathogic examination a diagnosis of IPEH was given. Case 2 is a 45-year-old man who presented with swelling on the dorsal aspect of the wrist. The cytologic diagnosis of giant cell tumor was made based on the presence of scattered spindled cells and multinucleate giant cells. The giant cells had various shapes like round or crescent and had 10-25 nuclei. The lesion was excised and a diagnosis of IPEH was rendered. CONCLUSION: These two cases highlight the varied cytomorphology of IPEH making the pinpoint diagnosis of this lesion difficult on cytologic smears.

Inflammatory pseudotumor of the spleen: a rare case diagnosed on FNAC.

Inflammatory pseudotumor (IPT) is a rare space-occupying lesion of unknown etiology that can mimic malignancy on clinic-radiological and pathological examination. We present a case of IPT of the spleen which was clinically suspected to be malignant. This case was initially suggested accurately on fine needle aspiration (FNA) of the mass and subsequently confirmed on histopathology of the resected specimen.

FNA in diagnosis of orbital lesions causing proptosis in adults.

The orbit is affected by a wide range of pathologic lesions, for which a morphologic diagnosis is needed to allow adequate therapy. With increasing use of fine needle aspiration (FNA) in diagnostic pathology, the procedure has been applied for the diagnosis of space occupying lesions of the orbit. We present the cytomorphological diagnosis on orbital FNA in adult patients presenting with proptosis. Records of seven adult patients who had presented with proptosis and had undergone orbital FNA were retrieved and analysed. FNA was performed from the palpable mass in six cases and under ultrasound guidance in one case. No complication during or after FNA was reported in any of the cases. Histopathological diagnosis was available in 5 cases. Out of the 7 cases, 3 were non-neoplastic (2 inflammatory lesions, 1 reactive lymphoid hyperplasia) and 4 were neoplastic (2 primary tumors and 2 secondary involvement). Both the primary tumors were non Hodgkin's lymphoma (NHL) B-cell type. Metastases included one case of uterine sarcomatoid carcinoma and one case of secondary involvement by extension of olfactory neuroblastoma. In all the neoplastic cases, cytological diagnosis corresponded with the histopathological diagnosis. It can be concluded that cause of proptosis among adults are different from those among children and include secondary malignancies. FNA is a simple and important preliminary diagnostic modality in the assessment of adult proptosis prior to any surgical intervention.

Refractory anemia as a presenting feature of microscopic polyangiitis: a rare vasculitis in children.

Microcytic anemia refractory to usual supplementation is an important clue to an alternative diagnosis. Accompanying pulmonary and renal involvement during the disease course suggests a multisystem disease. Small-vessel vasculitis should be suspected in any patient who presents with a multisystem disease that is not caused by an infectious or malignant process. Among these, antineutrophilic cytoplasmic antibodies (ANCA)-associated vasculitis is a distinct subclass involving anti-neutrophil cytoplasmic antibody (ANCA) as the common pathogenesis. Microscopic polyangiitis is a rare form of such vasculitis in children characterized by pulmonary-renal syndrome with pauci-imune rapidly progressive glomerulonephritis and a poor outcome.

Non-neoplastic salivary gland lesions: a 15-year study.

The spectrum of salivary gland lesions is wide and the relative incidence of neoplastic versus non-neoplastic lesions is variable in different studies. A series of non-neoplastic salivary gland lesions is reviewed to analyze their spectrum and their relative frequency. This is a retrospective study of salivary gland excisions and biopsies received in our department from January 1994 to December 2008. Routine hematoxylin and eosin-stained sections of all the salivary gland excisions and biopsies received were analyzed. Of the 393 salivary gland excisions and biopsies received, 216 cases were reported as non-neoplastic (55%) and formed our study group; 177 (45%) were neoplastic. Non-neoplastic lesions were more frequent in major salivary glands (65.7%) and submandibular gland was the most commonly involved (66.2%). Lip was the most frequent site (81.7%) for minor salivary gland lesions. Inflammation was the predominant pathological finding (49.5%), of which non-specific chronic sialadenitis constituted the majority (86.9%). Sialolithiasis was present in 22 cases (20.6%); all of these cases were of non-specific chronic sialadenitis. Cysts were second in frequency (36.6%), of which mucocele was the most common (54.5%). There were 5.6% cases of benign lympho-epithelial lesions, while normal salivary gland tissue was seen in 6.5% cases. Non-neoplastic salivary gland diseases are more common than neoplastic diseases and have a wide disease spectrum.

Chondroid syringoma: report of two cases in young patients.

Chondroid syringoma (CS) is a relatively rare cutaneous tumor arising from sweat glands. It usually presents in the head and neck area as an asymptomatic nodule. Although usually it presents in middle aged or older patients, we here present two much younger patients with CS, located over the nose and cheek areas, respectively.

Primary adenocarcinoma of ureter mimicking pyelonephritis.

Tumors of the ureter are rare. We present a case of primary mucinous adenocarcinoma of the ureter diagnosed as chronic pyelonephritis preoperatively. This tumor is postulated to arise from metaplastic glandular mucosa in response to chronic irritation of the urothelium.

Clinico-pathological spectrum of gallbladder disease in children.

AIM: Because of wide variation in clinico-pathological spectrum of gallbladder disease in children the world over, the data of gallbladder disease from this stone belt of India were analysed. METHODS: Children who underwent cholecystectomy over a period of 8 years January 2002-December 2009 were reviewed. RESULTS: Out of 7076 cholecystectomies, 56 (0.79%) were in children. Thirty-nine (69.6%) children were 11-16 years of age. Thirty-seven (66.07%) children were girls and nineteen (33.9%) were boys. In 12 (21.4%) children, cholecystitis was acalculus. Five (8.9%) children had associated haemolytic disease and 4 (7.1%) children had congenital anomaly in the form of choledochal cyst. Ultrasound findings were available in 44 cases and showed cholelithiasis in 36 cases. Twenty-two (39.3%) children had mixed cholelithiasis, 8 (14.2%) pigment cholelithiasis, 10 (17.8%) combined cholelithiasis and 4 (7.1%) patients had small concretions. Microscopically, changes of chronic cholecystitis were seen in 98.2% while 1.7% showed acute on chronic cholecystitis. There was single unusual case of cysticercus in the wall of the gallbladder. CONCLUSIONS: The frequency of gallstone disease is 0.79%. Nonhaemolytic type of cholelithiasis is more common than haemolytic type in this region. Presence of cysticercus in the gallbladder wall in one case was an unexpected finding.

Comparison of needle autopsy with conventional autopsy in neonates.

Tru-cut needle biopsy postmortems were compared with the standard conventional autopsy in neonates. Twenty-five fresh deceased neonates between 33 weeks of gestation to below 28 days of life were examined by both the techniques. Tissue collection by needle biopsy varied from 92% for liver to 20% for spleen while lung, brain, and kidney gave intermediate results. The cause of death could be determined in 17 cases (68%) by needle biopsy and in 24 cases (96%) by conventional postmortem. In 14 of the 17 cases (56%) cause of death could be determined by biopsy which was in full concordance with the findings of the full autopsy, whereas 3 (12%) cases showed only partial concordance and the diagnosis were discrepant in 32% cases. Needle autopsy missed diagnosis of various congenital malformations, which can be discerned only after meticulous gross examination. Needle autopsy is of value when permission for a complete autopsy cannot be obtained.

A comparative study of serum lipid profile and gallstone disease.

The diseased gallbladder is one of the commonest specimens submitted to the surgical pathology laboratory in North India. Obesity is associated with a linear increase in gallstone formation. It has been observed that the plasma lipoprotein profile of patients with gallstones differs markedly from that of healthy subjects. Serum lipid profile was done by enzyme kit method. All the gallstones received were categorized morphologically and examined biochemically. The age range of 200 cases was 13 to 77 years with a mean of43.75 +/- 13.39 years. There were 171 females (85.5%) and 29 males (14.5%) with male to female ratio of 1: 5.8. The stones containing both cholesterol and bile pigments were the most common (129 cases, 84.87%); while pure cholesterol stones were seen in 23 cases (11.50%) and pigment stones were infrequent (1 case, 0.65%). On lipidogram of patients in the study group, mean serum total cholesterol was 155.50 +/- 43.03 mg/dL, mean serum triglycerides was 100.49 +/- 45.23 mg/dL, mean HDL cholesterol was 46.71 +/- 15.20 mg/dL, mean LDL cholesterol was 87.94 +/- 36.85 mg/dL and mean VLDL cholesterol was 20.84 +/- 11.97 mg/dL. Serum total cholesterol values were significantly higher in patients older than 39 years as compared to patients < or =39 years (161.44 +/- 42.32 mg/dL vs. 145.79 +/- 32.96 mg/dL, p < 0.05). But the observed mean values in both of these subgroups were within the normal range i.e. <200 mg/dL. No significant difference was observed in the mean serum triglyceride values between male and female patients. The findings of this study did not indicate any role of serum lipid profile in the formation of gallstones. However the higher mean values of serum total cholesterol and serum triglycerides in patients older than 39 years of age may be explained by increasing age.

Steatocystoma multiplex: a report of two sporadic cases.

Steatocystoma multiplex is an autosomal dominant condition, though sporadic cases are also known, as in our cases. It is characterized by the presence of multiple non tender cystic nodules over the body, exuding syrupy fluid when excised. The lesions are usually seen around puberty and we present here two cases in middle aged with no family history of similar presentation.