Shiekh Aejaz Aziz

Primary adrenal lymphoma: Differential involvement with varying adrenal function

Primary adrenal Non-Hodgkins lymphoma is rare. The symptoms of the disease and response to treatment are variable depending on the type of lymphoma, tumor size, and presence of adrenal insufficiency. We report two cases of primary adrenal lymphoma who had varied presentations. One presenting with abdominal pain and weight loss was documented to have unilateral disease without any adrenal insufficiency and showed a good response to combination chemotherapy, while the second one had bilateral adrenal involvement with adrenal insufficiency and died after second chemotherapy. Functional adrenal involvement in lymphoma depends on the extent of involvement; patients with bilateral involvement almost always have adrenal insufficiency.

Giant cell carcinoma of the lung.

A 50-year-old non-smoking, hypertensive female, presenting with superior vena caval compression, was found to have giant cell carcinoma of the lung. She received intensive combination chemotherapy. However she died in the following 36 hours, as a consequence of refractory hypotension.

CARCINOMA OF THE GASTROESOPHAGEAL JUNCTION ASSOCIATED WITH ADULT POLYCYSTIC KIDNEY DISEASE

This article is also accessible online at: http://BioMedNet.com/karger Dear Sir, Adult polycystic kidney disease (APKD) is the commonest heritable renal disorder, inherited as autosomal dominant trait with variable expressivity and nearly 100% penetrance. A hypervariable marker and a gene resulting in APKD are close enough on the short arm of chromosome 16 to be inherited in approximately 95% of the cases [1]. Both renal and extrarenal (gastrointestinal, cardiovascular, genitourinary and neurological) manifestations of APKD are known [2]. The occurrence of malignancy in this second most common inherited homogenic disease was no higher than the expected incidence in Germany. However, a report of pooled data revealed an incidence tenfold higher than that expected in the general population. Approximately 50% of the cases of renal neoplasias have been reported in APKD. The vast majority of patients have renal cell carcinoma, a few others having papillary carcinoma, transitional cell carcinoma and sarcoid varaint of renal cell carcinoma [3]. Cholangiocarcinoma is anecdotally reported as extrarenal complication of APKD. Herein, we report a family with APKD, having 2 members (brother and sister) developing adenocarcinoma of the gastroesophageal junction (fig. 1, 2). The brother, 40 years

Case Report: Primary Squamous Cell Carcinoma of Kidney-An Unusual Malignancy

Primary Squamous Cell Carcinoma of the kidney is a rare clinical entity involving the upper urinary tract.These patients characteristically presents in advanced stage. This rare malignancy of the kidney lacks the characteristic clinical presentation of common renal cell carcinoma like hematuria, pain and a palpable mass. We report a 60 year old male who presented with a history of recurrent colicky right flank pain for 6 months duration which worsened in intensity and increased in frequency from last 1 month. He also had a history of untreated right renal calcular disease. On evaluation, he was found to have right renal mass with CT guided biopsy revealing Squamous cell carcinoma of kidney.

Malignant eccrine acrospiroma with nodal and bone metastasis

Malignant eccrine acrospiroma with nodal and bone metastasis Burhan Wani, Shiekh Aejaz Aziz, Mohmad Hussain Mir, Gull Mohammad Bhat, Abdul Rashid Lone Department of Medical Oncology, Sher-i-Kashmir Institute of Medical Sciences, Srinagar 190011, Kashmir, India. Correspondence to: Dr. Burhan Wani, Department of Medical Oncology, Sher-i-Kashmir Institute of Medical Sciences, Srinagar 190011, Kashmir, India. E-mail: burhan4187@gmail.com Acrospiromas are cutaneous tumors of sweat duct differentiation. Although various eccrine sweat gland tumours including benign acrospiroma are widely reviewed, malignant acrospiroma is rarely reported. Clinically, they resemble other cutaneous lesions and the primary treatment is wide local excision with or without lymph node dissection. The efficacy of adjuvant chemotherapy and radiation therapy requires further investigation.

Clinical profile of plasma cell leukemia at Tertiary Care Hospital in Kashmir, India

Introduction: Plasma cell dyscrasias represent 1.4–2% of all malignancies and among hematologic malignancies; it constitutes 10% of the tumors. Plasma cell dyscrasias are composed of multiple myeloma (MM), and primary and secondary plasma cell leukemia. Materials and Methods: Primary plasma cell leukemia (pPCL) is a rare and aggressive disease, representing 1–4% of plasma cell dyscrasias. Results: The prognosis of this is very poor with median survival of 8–11 months in different reported series. Discussion: We are reporting a study from our hospital over a period of 8 years and 9 months, in which pPCL was found in 1.8% of MM patients, with slight male predominance and earlier age than MM, and all had high disease burden with high lactate dehydrogenase (LDH), β2-microglobulin, and plasmacytosis. Conclusion: This disease had very aggressive behavior, especially with light chain lambda disease, and all patients succumbed within 8 months of treatment.