Sarcomas of the liver are rare. We report a case of intractable hypoglycemia secondary to a solitary fibrous tumor that underwent malignant transformation into a fibrosarcoma. A 70-year-old man presented with a hepatic mass and tumor-associated hypoglycemia which was resistant to medical management. Blood tests were remarkable only for elevated serum insulin-like growth factor (IGF)-2. The hypoglycemia resolved following resection of a solitary fibrous tumor surrounded by a high-grade fibrosarcoma. Real time reverse transcriptase polymerase chain reaction (RT-PCR) measured elevated levels of IGF2 mRNA in both the solitary fibrous tumor and the fibrosarcoma. Immunoblotting demonstrated a series of bands in the size range of pro-IGF2. Unfortunately, disseminated metastases developed 1 year later, concurrent with a recurrence of hypoglycemia, marked again by elevation of serum IGF2. Solitary fibrous tumors of the liver have a real risk of malignant transformation. The severity of the tumor-associated hypoglycemia may parallel the tumor burden and activity. The syndrome is the systemic effect of IGF2 secreted by the tumor. Surgery can treat the hypoglycemia syndrome and the underlying malignancy.
Acute liver failure continues to be associated with a high mortality rate, and emergency liver transplantation is often the only life-saving treatment. The short-term outcomes are decidedly worse in comparison with those for nonurgent cases, whereas the long-term results have not been reported as extensively. We report our center's experience with urgent liver transplantation, long-term survival, and major complications. From 1994 to 2007, 60 patients had emergency liver transplantation for acute liver failure. The waiting list mortality rate was 6%. The mean waiting time was 2.7 days. Post-transplantation, the perioperative mortality rate was 15%, and complications included neurological problems (13%), biliary problems (10%), and hepatic artery thrombosis (5%). The 5- and 10-year patient survival rates were 76% and 69%, respectively, and the graft survival rates were 65% and 59%. Recipients of blood group-incompatible grafts had an 83% retransplantation rate. Univariate analysis by Cox regression analysis found that cerebral edema and extended criteria donor grafts were associated with worse long-term survival. Severe cerebral edema on a computed tomography scan pre-transplant was associated with either early mortality or permanent neurological deficits. The keys to long-term success and continued progress in urgent liver transplantation are the use of good-quality whole grafts and a short waiting list time, both of which depend on access to a sufficient pool of organ donors. Severe preoperative cerebral edema should be a relative contraindication to transplantation.
The response of colorectal adenocarcinoma liver metastases to perioperative chemotherapy can be assessed histologically in partial hepatectomy specimens. Necrosis in this scenario may represent a lack of treatment effect or a therapeutic response to chemotherapy. This study sought to validate the histologic classification of necrosis into 2 types: usual necrosis (UN) representing an absence of treatment effect, and infarct-like necrosis (ILN) representing a therapeutic response to chemotherapy. Tumor regression grade (TRG) is a previously described prognosticating method that estimates tumor replacement by fibrosis. We incorporated ILN into a modified TRG (mTRG) and compared its performance as a prognostic factor against TRG. A retrospective clinical and histologic review was undertaken of all partial hepatectomies performed for colorectal liver metastases at our center between 2004 and 2010. Clinicopathologic features were compared between the 2 types of necrosis, including survival stratified by TRG and mTRG. A total of 109 cases were reviewed, with 46 patients receiving perioperative chemotherapy. ILN was identified in 12 cases, and all of these cases were associated with perioperative chemotherapy. ILN was significantly associated with perioperative treatment with bevacizumab. In patients receiving perioperative chemotherapy, those with ILN had superior disease-free survival compared with those with UN (P=0.047). mTRG1 to 2 scores were associated with significantly better survival compared with mTRG3 to 5 scores. In contrast, use of TRG did not demonstrate a significant difference in disease-free and overall survival. ILN represents a form of treatment effect and should be distinguished from UN. A modified grading system that incorporates ILN may enhance the prognostic utility of TRG.
Anaphylactic reactions were observed after Singapore’s national coronavirus disease 2019 (COVID-19) vaccination programme started in December 2020. We report the clinical and laboratory features of three patients in our institution who developed anaphylactic reactions after receiving the Pifzer BNT162b2 vaccine. IgM and IgG antibodies, but not IgE antibodies to the Pfizer BNT162b2 vaccine, were detected in all subjects. Similarly, mild to high elevated levels of anti-polyethylene glycol (PEG) IgG (1035–19709 U/mL, vs. vaccine-naive < 265 U/mL, vaccine-tolerant < 785 U/mL) and IgM (1682–5310 U/mL, vs. vaccine-naive < 1011 U/mL, vaccine-tolerant < 1007 U/mL) were detected in two out of three patients via commercial ELISA. High levels of serum anaphylatoxin C3a (79.0 ± 6.3 μg/mL, mean ± SD, vs. normal < 10 μg/mL) were observed in all three patients during the acute phase of the reaction, while tryptase levels, a marker of mast cell activation, were not elevated. Finally, one patient with the highest levels of anti-PEG IgG, IgM, and anti-Pfizer BNT162b2 IgG and IgM exhibited an enhanced Th2 cytokine serum profile during an acute reaction, with high levels of IL-4 (45.7 pg/mL, vs. vaccine-naive/tolerant < 2.30 pg/mL), IL-33 (86.4 pg/mL, vs. vaccine-naive/tolerant < 5.51 pg/mL) and IL-10 (22.9 pg/mL, vs. vaccine-naive/tolerant < 12.49 pg/mL) diminishing over time following corticosteroid treatment. Taken together, we propose these cases of anaphylaxis described are driven by a complement activation-related pseudoallergy (CAPRA), rather than classical IgE-mediated mechanisms.
At the Shouldice Clinic pre-operative weight loss is used prior to incisional hernia repairs. Mesh repair is selectively used, based on specific hernia characteristics. A series of 236 patients were reviewed and followed up for 36 months. Data were available on 188 patients (80%). There were 15 recurrences (8%). The number of obese patients was reduced from 67 (35.6%) to 25 (13.3%) through the weight loss program. The hernia diameter, gastrointestinal complications, and surgical site infection were significantly related to recurrence but not the type of repair, obesity, location, or previous recurrences. The risk factors of incisional hernias include size, intestinal complications and infections. A selective use has a comparable result to the exclusive use of mesh repair. Weight reduction has yet to be shown to affect the rate of recurrence, and further prospective studies are required.
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