Sonia Badwal

Pituitary metastasis from medullary carcinoma of thyroid: case report and review of literature

Pituitary is a rare site for metastases from thyroid cancer. Most reported cases have been of papillary and follicular carcinoma. Metastases from medullary thyroid carcinoma have not been reported. We report a case of intrapituitary metastasis from medullary carcinoma thyroid in a 38-year-old male, who had been operated for pituitary adenoma 5 years earlier. At the time of presentation in Nov 2006, he had visual field defects and a painless thyroid nodule. Further evaluation revealed medullary carcinoma thyroid, cervical and mediastinal lymphadenopathy, elevated serum calcitonin levels, and lobulated pituitary tumor. After surgical excision of thyroid and lymph node clearance, he underwent craniotomy and subfrontal excision of pituitary tumor. All the tumors were of identical histology, i.e., medullary carcinoma thyroid. Pituitary tumor was positive for calcitonin.

Clival Pituitary Adenoma with Acromegaly: Case Report and Review of Literature

The pituitary develops as a result of complex, intricate, and precise neuro-embryological events in the sixth to eighth weeks of gestation. Some ectopic cell rests can become adenomatous. Rarely, these cell rests in the clivus can be the site of formation of adenoma. Our patient, a 35-year-old parous woman, was being treated for acromegaly, and imaging studies revealed a clival mass lesion. Trans-sphenoidal excision was done and immunohistochemistry revealed the tumor to be a growth hormone-secreting tumor.

A case of reticulate acropigmentation of Kitamura: Dowling Degos disease overlap with unusual clinical manifestations

Reticulate hyperpigmentary disorders are a group of rare genetic pigmentary abnormalities which includes reticulate acropigmentation of Kitamura (RAPK), Dowling-Degos disease (DD), reticulate acropigmentation of Dohi (RAPD), Habers syndrome, and Galli-Galli disease. A 25-year-old male presented with asymptomatic dark-colored lesions on his hands and feet with light-colored skin lesions involving the trunk since three years. Dermatological examination revealed hyperpigmented macules in a reticulate pattern involving the dorsa of the hands and feet, front and sides of the neck, axillae, periorbital region, and groin. Multiple pits were present over both palms, with breaks in dermatoglyphics. He also had multiple nonacne facial scars predominantly on the nose and malar areas. The patient had overlapping features of RAPK and DDD. In addition, he also had hypopigmented macules and acneiform facial scars. Such an overlap of features of reticulate pigmentation has not been previously reported in the literature.

Hailey-Hailey disease with skin lesions at unusual sites and a good response to acitretin

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Rare case of subcutaneous mycosis with intrathoracic extension due to Chaetomium strumarium

A 47‐year‐old man presented with a 10‐year history of multiple lumps over his left upper arm and shoulder and the adjoining left side of his chest and upper back. His medical history included diabetes mellitus type 2. The patient was a farmer and used to lift sacks of grains and fertilizers onto his shoulders as part of his work, although he did not recollect any history of specific trauma. Skin biopsy revealed granulomatous reaction with Splendore–Hoeppli phenomenon, while periodic‐acid–Schiff and Grocott–Gomori stains confirmed fungal elements. Sabouraud agar grew Chaetomium species, and lactophenol blue mount confirmed the fungus as Chaetomium strumarium. Radiography and computed tomography of the chest revealed intrathoracic extension of the mycetoma. The patient responded well to treatment with oral Itraconazole. Subcutaneous mycosis due to C. strumarium is rarely reported in the literature, and the intrathoracic extension makes it an even rarer entity.

BK polyoma viral infection in renal allograft recipients.

BACKGROUND BK polyoma viral nephropathy (BKVAN) has emerged as a significant cause of renal allograft loss. The literature on BK viral infection from India is scarce. The study was therefore undertaken to evaluate impact of BK polyoma viral (BKV) infection on renal allograft recipients in Indian scenario from a service renal transplantation centre. METHODS Renal allograft recipients who underwent graft biopsy formed the part of this descriptive cross-sectional study group. The clinicopathological profile of the patients was analysed. The diagnostic modalities employed were histopathology, immunohistochemistry using antibody for Simian virus 40 large T antigen along with real time quantification of the BK viral DNA load in the urine and the serum. RESULTS One hundred forty seven renal allograft recipients were evaluated. 73.47 percent (108/147) patients presented with graft dysfunction and rest were protocol biopsies. There were 53 cases of rejection related diagnosis, 8 cases of graft pyelonephritis, 64 cases showed normal histology and rest exhibited miscellaneous causes. Nineteen percent (28/147) cases were positive for BKV DNA (viruria 26/147, 17.6% and viraemia 8/147, 5.44%. 3.4 percent (5/147) exhibited histological and immunohistochemical evidence of BKVAN. Nuclear enlargement, smudging and intranuclear inclusions along with plasma cell rich interstitial nephritis were important features observed on histopathology. Concomitant acute rejection was seen in 4/5 cases of BKVAN. All cases of BKVAN exhibited viraemia (> 2500 copies/mL), though cut-off values could not be defined statistically due to small sample size. Positive statistical correlation was observed between use of anti-thymocyte globulin (induction therapy and/or treatment of steroid resistant rejection, Pearson ×(2) value 6.9, P=0.008) and rejection episodes (Pearson ×(2) value 9.8, P = 0.007) with BKV infection. CONCLUSION BK polyoma nephropathy should be added to the list of differential diagnosis considered for a renal allograft dysfunction. Renal biopsy remains the gold standard for diagnosis supplemented by non-invasive molecular techniques for screening and monitoring of BKV infection.

Unusual case of pulmonary renal syndrome with autopsy findings

Scleroderma renal crises (SRC) is a serious complication of systemic sclerosis whose prognosis remains serious despite management with angiotensin-converting enzyme inhibitors, antihypertensives and dialysis. Pulmonary renal syndrome (PRS), characterised by diffuse alveolar hemorrhage (DAH) and SRC, is rare and carries a grave prognosis. This case report discusses the clinicopathological features of a 43-year-old male presenting with severe hypertension and rapidly progressive renal failure who subsequently developed DAH and died. The clinical course, exhaustive investigative work-up and autopsy findings led to a diagnosis of diffuse systemic sclerosis with PRS subcategorized into PRS with thrombotic microangiopathy. The index case came without a prior diagnosis of systemic sclerosis, thereby posing a serious diagnostic challenge and management issues.

Evaluation of cases of membranoproliferative glomerulonephritis according to newer classification: A retrospective record-based study

Background Membranoproliferative glomerulonephritis (MPGN) has traditionally been classified on electron microscopy (EM) into different types based on the location of the immune complexes. Sethi et al. subsequently suggested a more relevant etiology-based and clinically useful classification based on immunofluorescence. Methods In this retrospective study, 18 diagnosed cases of MPGN over a one-year period for which direct immunofluorescence (DIF) study results were available, were selected. Cases without archived records of immunofluorescence photographs/reports were excluded. Histological diagnosis of MPGN was confirmed and DIF results were analyzed with reference to antibodies to IgG, IgA, IgM, C3, C1q, kappa, and lambda light chains. Results Evaluation of cases revealed 8 males and 10 females with age range from 11 to 66 years. Fifteen cases presented with nephrotic syndrome. On evaluation, 88.89% cases (16/18) were immune complex mediated while two (11.11%) were of complement mediated type of MPGN. Among immune complex-mediated cases, a single case of monoclonal gammopathy associated or light chain mediated MPGN was present. Conclusion The classification described by Sethi et al. is easy to use since it relies on DIF instead of EM which is not readily available. Most of the cases were immune complex mediated whereas incidence of complement mediated MPGN, that is, C3 glomerulopathy was low (11.11%). Application of the new classification allows more relevant categorization of cases based on etiology and without the requirement of EM.

Anti-neutrophil cytoplasmic antibody negative crescentic paucimmune glomerulonephritis in a case of scleroderma with systemic lupus erythematosus overlap

Renal Involvement in scleroderma is a known problem and the manifestations are well described. Renal involvement in systemic lupus erythematosus (SLE) is also well known. However, in scleroderma and SLE overlap syndrome, the renal findings may vary being a combination of features of immune complex mediated glomerulonephritis as well as thrombotic microangiopathy. We report a case in which the renal manifestation in such a situation was of a focal necrotising pauci-immune glomerulonephritis with crescents, anti-neutrophil cytoplasmic antibody negative. To the best of our knowledge, such manifestations have not been described before. Renal dysfunction in a normotensive setting in such a case should direct one towards evaluation for other causes and should prompt a kidney biopsy. This would be valuable in delineating the pathological process in the kidney and would help in guiding therapy.

Immuno-histological assessment of sub-clinical acute and borderline rejection in renal allograft recipients: Data from a transplant center in India

This single-center study was carried out on living related and unrelated renal transplant recipients (RTRs) to evaluate the usefulness of surveillance biopsies in monitoring stable renal allografts using immuno-histological markers for immune-activation. This is a prospective, longitudinal study. Protocol biopsies of 60 RTRs with stable graft function were evaluated at three, six and 12 months post-transplant. Immuno-histological evaluation was carried out using immune-activation markers (perforins, granzyme and interleukin-2R), phenotypic markers (CD-3 and CD-20), viral markers and C4d. The demographic and clinical profile was recorded for each patient. All cases of acute sub-clinical rejection (SCR) were treated and borderline SCR cases were followed-up without treatment. SCR at three and six months post-transplant was evident in 16.7% and 3.7% of RTRs, respectively. Positive statistical association of SCR was seen with HLA-DR mismatches, whereas patients receiving induction therapy and tacrolimus-based immunosuppression exhibited a lower incidence of SCR. T cell phenotype with persistent expression of immune-activation markers exhibited positive statistical association with interstitial fibrosis and tubular atrophy at 12-month follow-up biopsy. The mean creatinine levels were significantly lower in the protocol biopsy group than the non-protocol biopsy group. No significant difference was found between the mean creatinine levels of the SCR group after treatment and the non-SCR cases within the protocol biopsy group. Early treatment of sub-clinical acute rejection leads to better functional outcomes. However, persistent immune-activation is associated with chronicity and may have implications on long-term graft survival.