HighlightsDesmoid tumors are benign but lead to morbidity and mortality due to mass effect.Histologically, desmoid tumors can masquerade as other neoplastic processes.Chemo- and radio-therapy are useful, but surgery is the cornerstone of therapy.The recurrence rate, even after treatment, is high.
INTRODUCTION: Diabetic hepatosclerosis (DH) is an uncommon type of hepatic injury associated with poorly controlled diabetes mellitus (DM). Unlike steatohepatitis or glycogenic hepatopathy, DH is a form of end-organ damage due to microvascular injury. This case illustrates a patient with advanced DM who presented with cholestatic liver injury and histologic findings of DH. CASE DESCRIPTION/METHODS: A 34 yo woman with poorly controlled type 1 DM (HgA1C 17%) complicated by diabetic nephropathy presented with one month of generalized pruritis. Symptoms were most severe after hot showers and at night. She denied rash, fevers, unusual exposures, ingestions, or new medications. On exam, she appeared well, had no visible skin lesions, no scleral icterus, and a soft abdomen without organomegaly. Labs were notable for ALP 612 U/L, ALT 205 IU/L, AST 109 IU/L, TB 0.9 mg/dL, WBC 8.3 K/uL, PLT 306 K/uL. Abdominal ultrasound revealed a normal appearing liver and biliary ducts. Ultrasound-guided liver biopsy showed perivenular and perisinusoidal fibrosis without inflammation, steatosis, or bile duct injury. These findings were consistent with DH. After one year of aggressive glycemic control, her pruritus resolved. Her HgA1C was 7.6%, ALP 313 IU/L, ALT 32 IU/L, AST 29 IU/L. DISCUSSION: While non-alcoholic fatty liver disease (NAFLD) and glycogenic hepatopathy are more commonly seen in patients with advanced DM, DH is a distinct form of microangiopathic liver injury characterized by dense perisinusoidal fibrosis (Figure 1), lack of steatosis (Figure 2), and basement membrane deposition of laminin and type IV collagen1,2. DH can be seen in both type 1 and type 2 DM, and rarely occurs in the absence of other microvascular complications. DH is characterized by a predominantly elevated ALP, suggesting a primary bile duct or canalicular injury. Diabetics with hypertension seem to be at increased risk for DH3. The long term consequences of DH remain unclear. In our patient, a decrease in HgA1c level resulted in normalization of AST and ALT levels, a marked reduction in ALP, and resolution of pruritus.
INTRODUCTION: Cronkhite Canada syndrome (CCS) is a rare, acquired polyposis syndrome associated with serious complications and a high mortality rate. Awareness regarding presenting symptoms, endoscopic findings, and pathologic features leads to early diagnosis and treatment, and improved survival. CASE DESCRIPTION/METHODS: 68 yo female with a history of alopecia totalis presented with 6 months of non-bloody diarrhea, 30 lb weight loss, and dysgeusia. Physical examination revealed complete alopecia, pitting lower extremity edema, and thinning and splitting of the fingernails. Diagnostic workup yielded marked hypoalbuminemia (albumin 1.1 g/dL), positive SS-a antinuclear antibody (titer 1:160), and elevated serum total IgG level (1,750 mg/dL). CT was notable for a thickened stomach with edematous rugae, and focal segmental colitis involving the cecum and ascending colon. Push enteroscopy showed diffusely edematous mucosa in the gastric antrum and duodenum with denuded appearing vili throughout the small bowel. Colonoscopy revealed diffuse polyposis throughout the entire colon and terminal ileum, with edematous underlying colonic mucosa. Biopsies of affected areas showed diffuse cystic dilation, edema of the lamina propria, and presence of plasma cells, compatible with CCS. The patient was initiated on total parenteral nutrition, prednisone 40 mg daily, and Imuran 75 mg daily. In follow up, she reported complete resolution of dysgeusia, reduction in diarrheal symptoms, weight gain, and improved energy level. Serum albumin level improved to 2.92 g/dL. DISCUSSION: Cronkhite Canada syndrome is a sporadic, acquired polyposis syndrome characterized by diffuse gastrointestinal polyposis and ectodermal features. It carries a 5-year mortality rate of 55% despite therapy, due to a high rate of serious complications, including malabsorption, infection, heart failure, and GI bleeding. Patients are at high risk of gastric and colorectal malignancies (10.4% and 25%, respectively), necessitating annual screening examinations. A combination of nutritional support and immunosuppression with prednisone (tapered dosing) and imuran (2 mg/kg) helps in achieving clinical remission and avoiding relapse. Expedited diagnosis and application of therapy has led to improvement in patient survival and achievement of clinical response. Therefore, improved clinician awareness of disease manifestations, pathologic findings, and treatment regimen is necessary to modify the disease course for this syndrome.
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