Autophagy is involved in the pathological process of motor neuron death in amyotrophic lateral sclerosis (ALS). We have generated a novel double transgenic (DTg) mouse line by mating a green fluorescent protein (GFP)-fused microtubule-associated protein 1 light chain 3 (LC3) transgenic (LC3-Tg) mouse and a G93A mutant human Cu/Zn superoxide dismutase (mSOD1) transgenic (mSOD1-Tg) mouse. In vivo imaging of autophagy with these novel DTg mice was conducted at 10 (presymptomatic), 17 (early symptomatic) and 19 (late symptomatic) weeks of age. Fluorescence imaging analysis revealed a strong fluorescent signal in vivo over the T₃-S₁ level at 17 and 19 weeks of age only in the DTg mice. Ex vivo autophagy imaging of spinal cord sections (20 μm) also showed a progressive increase of the fluorescence signal from 17 to 19 weeks in DTg mice in the anterior horn at the L₄-₅ level, and the fluorescence signals were clearly observed in the gray matter of the spinal cord with a progressive increase of the signal and decreases in large motor neurons. Protein gel blot analysis revealed maximum LC3-I and LC3-II expressions at 19 weeks, consistent with the results from the in vivo autophagy imaging experiment. This method could also be applied as a unique tool for clarifying the role of autophagy, and to monitor the pathologic processes involving autophagy not only in ALS, but also other neurological diseases.
Rationale:Sarcomatous intrahepatic cholangiocarcinoma is a rare histological variant of cholangiocarcinoma (ICC). Previous medical literature has not mentioned the prevalence of this kind of disease, but a poorer prognosis than that of ordinary ICC was indicated. The diagnosis of the sarcomatous ICC is established on histopathological and immunohistochemical examinations. In this article, we present a new case of a patient with sarcomatous ICC who had no radiographic sign of intrahepatic tumor preoperatively.Patient concerns:A 63-year-old man was noted with cholecystolithiasis and right upper abdominal pain. Liver function was within normal limits, although the gamma-glutamyl transpeptidase level was elevated. Serum carbohydrate antigen 19-9 level was elevated. Radiography showed atrophy of the left lobe of the liver, high-intensity signals on T1 weighted images, and low/high-intensity signals on T2 weighted images in hepatic ducts.Diagnoses:The preoperative diagnoses were hepatolithiasis, choledocholithiasis, and cholecystolithiasis.Interventions:Exploratory laparotomy, adhesion release, cholecystectomy, choledocholithotomy, and T tube drainage were performed. During the surgery, an ill-defined tumor was detected on the atrophic left lateral lobe of the liver. Hepatic left lateral lobectomy was performed to remove the mass.Outcomes:The final diagnosis of sarcomatous ICC was made by histopathology after surgery. No evidence of local recurrence or distant metastasis was noted on imaging during follow-up.Lessons:Although rare, sarcomatous ICC does exist in patients presented with cholecystolithiasis and liver atrophy. Surgeons should be aware of the existence of sarcomatous ICC due to the poor prognosis. We recommend that multidisciplinary approaches may be key to improve prognosis, including adjuvant chemotherapy or radiotherapy.
We present the case of a 66-year-old woman with a huge gastrointestinal stromal tumor of the stomach that traversed her upper abdomen. The predominant abdominal sign was a huge, palpable mass, but there were no other distinctive findings in her physical examination or her routine blood workup, including biochemical markers. It was difficult to judge the origin of the mass upon imaging. Furthermore, radiological findings revealed that the mass had a complex relationship with many major blood vessels. An exploratory laparotomy revealed a huge tumor protruding from the anterior wall of the stomach fundus, on the lesser curvature of the stomach, measuring approximately 21 × 34 × 11 cm in diameter and weighing 5.5 kg. A complete resection was performed and the tumor was characterized on immunohistochemistry as a gastrointestinal stromal tumor of the stomach. Preoperative diagnosis of gastrointestinal stromal tumors can be difficult, and we hope that the presentation of this rare case and literature review will benefit other diagnosing clinicians having similar problems.
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