A rare case of acute pancreatitis due to very severe hypertriglyceridemia treated with subcutaneous insulin and lipid lowering drugs.

The diagnosis of acute pancreatitis in a patient requires the presence of two of the following three criteria: (I) acute onset of persistent, severe; (II) epigastric pain often radiating to the back, elevation in serum lipase or amylase to three times or greater than the upper limit of normal; (III) characteristic radiographic evidence hypertriglyceridemia is a potential cause of acute pancreatitis when levels are greater than 1,000 mg/dL. Very severe hypertriglyceridemia is classified as levels above 2,000 mg/dL. Management includes targeting pancreatitis with intravenous fluids, pain control, and nutritional support. While apheresis with therapeutic plasma exchange is a known option for severe hypertriglyceridemia, we present a rare case with management with intravenous fluids, subcutaneous insulin, statins, and fibrates in a patient with a triglyceride level of 12,234 mg/dL who presented with severe epigastric pain radiating to her back.

A Rare Case of Stage IV Basaloid Squamous Cell Cancer with Intrapulmonary and Brain Metastases.

BACKGROUND Metastatic basaloid squamous cell carcinoma is a fatal, high-grade variant of squamous cell carcinoma that is extremely rare in the oral cavity. We present a rare case of metastatic basaloid squamous cell carcinoma arising from the hypopharynx with pulmonary and brain metastases. Recognizing this diagnostic subtype is of critical importance due to the aggressive nature and high incidence of recurrence, lymph node metastases, and mortality. CASE REPORT A 42-year-old male arrived at the Emergency Department reporting a 1-week headache. Six months prior, he reported throat pain and neck swelling. Triple endoscopy revealed a large ulcerative tumor. A carbon dioxide laser procedure debulked and removed the mass. Incisional biopsy with histopathology was consistent with invasive basaloid squamous cell carcinoma. Computed tomography (CT) of the neck with contrast demonstrated bilateral cervical level II/III necrotic adenopathy, and CT chest with contrast demonstrated bilateral pulmonary nodules. The patient completed chemoradiation therapy with cisplatin; however, repeat CT chest revealed enlarging intrapulmonary metastases. CT brain without contrast demonstrated a central brainstem lesion. The patient started treatment with pembrolizumab. On day 14 of treatment, he presented to the Emergency Department again for headache. MRI of brain with contrast demonstrated a new lesion with vasogenic edema. Intravenous dexamethasone was started and the decision to pursue stereotactic radiosurgery was made. CONCLUSIONS The diagnosis of basaloid squamous cell carcinoma in the setting of intrapulmonary and brain metastases is an extremely rare, high-grade bimorphic aggressive variant of squamous cell carcinoma that needs to be histopathologically differentiated from other tumors. Given its high mortality rate and poor prognosis the decision to pursue further treatment versus aggressive palliative care should be discussed.

Splenic Marginal Zone Lymphoma in the Setting of Noncirrhotic Portal Hypertension.

We present a case of a 65-year-old Hispanic man with a history of disseminated cutaneous coccidioidomycosis who presented to the emergency room for progressively worsening abdominal pain associated with shortness of breath. The patient was found to have pleural effusion and moderate ascites on physical examination. Abdominal ultrasound and computed tomography scan were consistent with moderate ascites and portal hypertension but negative for both liver cirrhosis and for venous or arterial thrombosis. Cytology of ascitic fluid was suggestive of portal hypertension and was negative for infection. Subsequent, thoracentesis was suggestive of exudative effusion and also negative for infection. Liver biopsy confirmed the absence of cirrhosis. Complete blood count indicated pancytopenia, whereas bone marrow biopsy and flow cytometry were suggestive of marginal zone lymphoma (MZL). Clinically, the patient's shortness of breath was resolved by thoracentesis and paracentesis; however, his abdominal pain persisted. A diagnosis of idiopathic noncirrhotic portal hypertension in the setting of splenic MZL was made. The patient was transferred to a higher level of care for splenectomy; however, he missed multiple appointments. Since discharge, the patient has been seen in the outpatient setting and states that he is controlling his disease with diet and exercise; however, he continues to complain of intermittent shortness of breath with exertion.

Lymphangioleiomyomatosis: A review.

Lymphangioleiomyomatosis (LAM) is a rare disease, of unknown etiology, affecting women almost exclusively. Microscopically, LAM consists of a diffuse proliferation of smooth muscle cells. LAM can occur without evidence of other disease (sporadic LAM) or in conjunction with tuberous sclerosis complex (TSC). TSC is an autosomal dominant tumor suppressor gene syndrome characterized by seizures, mental retardation, and tumors in the brain, heart, skin, and kidney. LAM commonly presents with progressive breathlessness or with recurrent pneumothorax, chylothorax, or sudden abdominal hemorrhage. Computed tomography (CT) scans show numerous thin-walled cysts throughout the lungs, abdominal angiomyolipomas, and lymphangioleiomyomas. No effective treatment currently exists for this progressive disorder. The prevalence of lymphangioleiomyomatosis is probably underestimated based on its clinical latency and the absence of specific laboratory tests. With the utilization of international LAM data registries the "classical" picture of the disorder appears to be evolving as a larger number of patients are evaluated. An increased awareness of LAM and its common clinical presentation may advance the development of new therapeutic strategies and reduce the number of mistakenly diagnosed patients.