Primitive neuroectodermal tumors (PNETs) are small, round-cell tumors of neural crest origin classically found in the central nervous system (CNS) but more recently characterized in the periphery. Peripherally located PNETs (pPNETs) are members of the Ewing's sarcoma family of tumors (EFTs). Renal localization of pPNETs is very rare, is found in young adults, and is characterized by an aggressive clinical course and poor prognosis. We present the case of a young man with renal pPNET characterized by psuedorosette formation, cluster of differentiation 99 (CD 99+), focally positive for neuron-specific enolase (NSE), with cytogenetic findings of the translocation t(11;22)(q24;q12) and the unique abnormality of trisomy 7. To our knowledge, we report the first case of trisomy 7 and PNET.

Calciphylaxis is a rare disorder characterized by calcification of small and medium-size arteries, causing thrombosis, tissue necrosis, and painful, nonhealing skin ulcerations. The preponderance of cases occurs in patients undergoing dialysis due to endstage renal disease. Prognosis is generally poor, with one-year mortality ranging from 45% to 80% 1,2. A recent report noted that even among nondialysis patients, renal function was moderately to severely impaired in 69% [glomerular filtration rate (GFR) < 40 ml/min] 1. Autoimmune or inflammatory conditions were present in 60%, including systemic lupus erythematosus (SLE), polymyositis, sarcoidosis, hepatitis, ulcerative colitis, rheumatoid arthritis, Sjögren's syndrome, and pemphigus. Various other risk factors are documented, including hyperparathyroidism, elevated serum calcium and phosphate, hypoalbuminemia, obesity, diabetes mellitus, and female sex, and use of warfarin, vitamin D3 analogs and calcium-containing phosphate binders 1,2. Couto, et al described a patient with secondary hypoparathyroidism due to vitamin D deficiency, with low calcium/phosphorus product, who developed calciphylaxis in the absence of endstage renal disease 3. Case reports of calciphylaxis with normal calcium/phosphorus product as well as normal renal function are limited. We describe a case of calciphylaxis in the setting of normal renal function, in a patient with SLE, secondary hyperparathyroidism due to vitamin D deficiency, and normal calcium/phosphorus product. A 76-year-old African American woman presented to hospital complaining of worsening severe pain, weight loss, fever, night sweats, and chills. Three months previously she first noted painful lesions near her hips and inner thighs. Initial outpatient punch biopsies showed lipophagic fat necrosis without calcification, the differential favoring lupus panniculitis. The wounds showed limited healing on a course of topical and oral steroids. Medical history included hypertension, atrial fibrillation, SLE (antinuclear antibodies 54 U/ml, ribonucleoprotein 245 AU/ml, anti-Smith 115 AU/ml), breast cancer status post-lumpectomy and radiation therapy, diverticulosis, and osteoarthritis. Surgical history included bilateral total knee arthroplasties. Medications were alendronate, dicyclomine, fluoxetine, furosemide, gabapentin, hydrocodone/acetaminophen, lidocaine oint

Klinefelter's syndrome is characterized by hypogonadism and infertility and commonly has an XXY karyotype. Within the population of men with this disorder, there is an increased incidence of venous thromboembolic disease. Although the precise mechanisms underlying this prothrombotic state have not been elucidated, it is thought that the increased incidence of thromboembolism is associated with a hypofibrinolytic state secondary to androgen deficiency. We present the case of a 26-year-old man with Klinefelter's syndrome who had recurrent episodes of deep venous thrombosis and pulmonary embolism while undergoing therapeutic anticoagulation. Coagulation studies were significant for the heterozygous mutations of MTHFR-677C>T and 1298A>C gene and hyperhomocystenemia. Our aim is to raise awareness of this association and discuss management for these patients.