Recurrence of primary hyperoxaluria: an avoidable catastrophe following kidney transplant.

Primary hyperoxaluria is a rare autosomal recessive disease due to deficiency of an oxalate-metabolizing liver enzyme, which results in nephrolithiasis and renal failure. Concomitant liver and kidney transplant is recommended as isolated kidney transplant is inevitably complicated by recurrence of the disease. We present a 25-year-old man with end-stage nephrolithiatic renal disease who underwent bilateral nephrectomy, followed by kidney transplantation. There was progressive worsening of kidney function two weeks post transplant. Review of nephrectomy and transplant kidney biopsy showed abundant calcium oxalate crystals and further workup revealed hyperoxaluria, which was previously unsuspected. Later he developed fever, breathlessness, hemiparesis and died 10 weeks after transplant. Autopsy revealed multi-organ deposits of oxalate crystals as well as widespread zygomycosis. This case emphasizes the need for careful pre-transplant evaluation of patients with renal calculus disease in order to exclude primary hyperoxaluria.

Isolated breast mucormycosis.

Mucormycosis is unusual in surgical practice. Awareness of the classical findings leads to early detection. Excisional therapy whenever possible along with systemic antifungal treatment is the key to successful outcome. A 70 year old female, a known case of diabetes mellitus and chronic obstructive pulmonary disease, on inhalational steroids and oral hypoglycemic agents, presented to us with complaints of sudden onset pain, redness and swelling of left breast. A diagnosis of severe mastitis was made and a release incision was taken. The entire breast became gangrenous in next 24 hours and simple mastectomy had to be performed as a life saving measure. Histopathology revealed mucormycosis of breast. To the best of our knowledge this is the first reported case of mucormycosis of breast in the English literature.

Sino-nasal epithelial tumours: a pathological study of 69 cases.

BACKGROUND: Epithelial neoplasms are uncommon lesions affecting the sino-nasal tract. There are hardly any reports in the Indian literature. AIM: To study the incidence, mode of presentation and histological types of sino-nasal epithelial tumours in the surgical pathology material. SETTING AND DESIGN: Retrospective retrieval of all sino-nasal tumours and analysis of epithelial tumours. MATERIALS AND METHODS: All sino-nasal epithelial tumours, biopsied or surgically excised over a period of ten years, were studied. The tumours were classified as benign or malignant. The histology was correlated with the clinical presentation and investigations. RESULTS: In ten years, there were 120 sino-nasal tumours representing 0.14% of all the surgical specimens received. Sixty-nine epithelial tumours (59.2%) outnumbered the non-epithelial tumours and were diagnosed on the basis of histopathology. Twenty were benign and 49 malignant; occurring predominantly in males. Benign lesions included four squamous papillomas and 16 inverted papillomas, with recurrence in three inverted papillomas (21%). Squamous cell carcinomas were the commonest among malignant tumours and four of these were associated with inverted or cylindrical cell papilloma. The second most frequent malignant tumour was adenoid cystic carcinoma with eight cases. Other rare types included the variants of squamous carcinoma, adenocarcinomas of the non-enteric type, muco-epidermoid carcinoma and undifferentiated carcinomas. CONCLUSION: Sino-nasal epithelial tumours are rare lesions, with male preponderance. Inverted papillomas and squamous cell carcinomas are the most frequent neoplasms.

Mullerian adenosarcoma of the uterine cervix.

Sarcomas in the uterine cervix are rare, the incidence being 0.5% to 1% of all cervical malignancies. This is a report of cervical mullerian adenosarcoma, which was encountered in a hysterectomy performed for prolapse. The tumor was composed of benign glandular elements and malignant stromal component, thus justifying its nomenclature. We wish to emphasize the distinctive morphological features of this rare cervical tumor.

Isolated massive splenic peliosis.

We report a patient with isolated splenic peliosis. She presented with massive splenomegaly; contrast-enhanced CT scan showed multiple hypodense, well-circumscribed shadows in the spleen. Splenectomy specimen showed multiple cysts with white fibrous walls ranging from 1-4 cm in size, containing gelatinous fluid. The patient is asymptomatic at 6 months' follow up.

Carcinoma arising within fibroadenoma--a case report.

A rare case of ductal carcinoma-in-situ in multiple fibroadenomas of the breast in a 43 year old female is reported.

Xanthogranulomatous pyelonephritis presenting as emphysematous pyelonephritis: a rare association.

Xanthogranulomatous and emphysematous pyelonephritis are two rare variants of pyelonephritis. Their combined occurrence is a very rare condition, which has been documented in our case.

Xanthogranulomatous oophoritis--a case report.

This is a case of a 25 year old unmarried women who presented with intermittent fever and lower abdominal pain. Laparotomy revealed a large cystic left sided tuboovarian mass adherent to surrounding structures and containing foul smelling fluid. Microscopy showed extensive replacement of the ovary by a chronic inflammatory exudate composed predominantly of foamy macrophages.

Renal lesions in AIDS: a biopsy and autopsy study.

We studied renal lesions at biopsy (20 cases) and at autopsy (21 cases) among patients with the acquired immune deficiency syndrome (AIDS). Nephrotic syndrome with concomitant renal insufficiency was most common indication for biopsy. 85 percent of biopsies showed features of HIV associated nephropathy (HIVAN) which include: Focal segmental glomerulosclerosis (FSGS), glomerular collapse and mesangial hyperplasia. These glomerular changes were always accompanied by tubular microcysts and ultrastructurally, tubuloreticular inclusions (TRI) within the glomerular endothelium were often noted. Changes of HIVAN were also seen in two cases who were HIV negative at the time of biopsy but were positive on repeat testing. Minimal change disease, mesangiocapillary glomerulonephritis and diffuse proliferative lupus nephritis were other biopsy lesions. Autopsy findings were HIVAN (33 percent), tubular necrosis and opportunistic infections. We conclude that HIVAN is a distinct clinicopathologic entity that may sometimes be the first manifestation of the underlying disease state.

Renal arterial aneurysm--an incidental finding at autopsy.

Herein we describe a rare case of saccular renal artery aneurysm seen as an incidental autopsy finding in an elderly, hypertensive female. The aneurysm was seen as a small exophytic mass with calcified wall and lumen occluded by recanalized thrombus.

Ebstein's anomaly--an autopsy study of 28 cases.

Twenty eight autopsy specimens of Ebstein's anomaly were studied in order to evaluate the morphologic features of the abnormal tricuspid valve. All cases showed marked dilatation of the original tricuspid annulus, a normally positioned anterior leaflet and variable downward displacement of the posterior and septal leaflets. Sixteen cases showed a very large anterior leaflet. All three leaflets showed dysplastic features and a wide range of anatomic abnormalities in the valve and valve apparatus. A thin walled atrialised right ventricle was present in nine cases. Associated cardiac anomalies were seen in 21 cases, the commonest being an atrial septal defect (17 cases).

Tuberculosis of the renal artery.

A young female who underwent nephrectomy for renovascular hypertension was diagnosed on histology to have tuberculosis of the renal artery. This was an isolated finding as there was no tuberculous infection elsewhere including tissues in the vicinity of the vessels. A survey of literature did not yield any reports of tuberculous renal arteritis, making this the first such case.

Hydatid cyst of the tibia.

A case of hydatid cyst of the tibia, which manifested as a pathologic fracture is being reported. Pain and swelling of left lower limb with inability to bear the weight were the main features. Tender swelling was also noted at the upper and middle third of tibia. Open biopsy revealed the hydatid cyst wall and scolices of Echinococcus granulosus. Albendazole treatment was followed by curettage and bone grafting.

Fine needle aspiration cytology (FNAC) in the evaluation of acute renal allograft rejection.

310 Fine needle aspirations biopsies (FNAB) were performed in sixty-five live related renal donor transplant recipients in a prospective study over 2 years. 82.8% of FNAB were adequate for opinion and there were no complications after the procedure. Methodology used and interpretation of FNACs was as described by Von Willebrand and Hayry. The procedure of FNAC had a sensitivity of 83.3%, specificity of 98.3% and accuracy of 96.1%, and statistically significant (p < .001) correlation was noted between FNAC and Needle biopsy. The increment in lymphoblasts, lymphocytes and score above 3 was suggestive of acute cellular rejection. Increment in monocytes and macrophages above 1% in addition to other cells, was suggestive of acute vascular rejection. FNAC was found to be a safe, simple and easy procedure with high specificity and sensitivity.