Dural Metastasis without Subdural Hematoma or Subdural Fluid Collection in a Patient with Signet Ring Cell Gastric Adenocarcinoma.

BACKGROUND Signet ring cell (SRC) gastric adenocarcinoma is an aggressive histotype associated with poor prognosis, especially in advanced gastric cancer. Dural metastasis is rarely described in the literature, and clinical manifestations are generally related to subdural hematoma. Here we present a case of advanced SRC gastric cancer with dural neoplastic involvement in the absence of subdural hematoma or subdural fluid collection. CASE REPORT A 39-year-old woman presented with multiple episodes of confusion and headache. She had a history of SRC gastric adenocarcinoma that had been treated with neoadjuvant chemotherapy and total gastrectomy without evidence of disease relapse at follow-up. During hospitalization, she experienced persistent drowsiness and frequent generalized seizures that were nonresponsive to antiepileptic drugs. Brain computed tomography showed a dural right parafalcine nodular lesion suggestive of metastasis, and an SRC presence was detected in a cerebrospinal fluid sample. Cerebral magnetic resonance imaging showed isolated diffuse dural neoplastic involvement without evidence of subdural hematoma or subdural fluid collection. We considered palliative treatment with intrathecal chemotherapy, but it was not carried out because of clinical worsening and subsequent death. CONCLUSIONS In the very few case reports in the literature, dural metastasis in advanced gastric cancer is mainly associated with subdural hematoma. In our case, the absence of any subdural effusion, which is an even rarer condition, along with an unusual clinical presentation dominated by generalized seizures represented a diagnostic challenge. Given the aggressive course of the disease, a rapid diagnosis could allow a faster specific treatment to relieve a patient's symptoms.

Different NT-proBNP circulating levels for different types of cardiac amyloidosis.

AIM: Several studies suggest that the N-terminal fragment of pro-brain natriuretic peptide levels are quite different in wild-type transthyretin (TTR)-related amyloidosis (ATTRwt) and mutated TTR-related amyloidosis (ATTRm) compared with immunoglobulin light-chain cardiac amyloidosis. Our aim was to test this hypothesis in a cohort of patients with different types of cardiac amyloidosis. PATIENTS AND METHODS: Seventy patients with ATTRwt, ATTRm, and light-chain cardiac amyloidosis matched for left ventricular (LV) mass index were studied by standard echocardiography, tissue Doppler imaging, and plasmatic cardiac biomarkers. RESULTS: Despite similar LV mass and renal function, patients with ATTR cardiac amyloidosis showed lower levels of N-terminal fragment of pro-brain natriuretic peptide than do light-chain amyloidosis ones, especially when expressed as a function of LV mass index. CONCLUSION: Amyloidogenic light-chain-derived fibrils induce more severe myocardial dysfunction in light-chain amyloidosis than in ATTR, despite similar myocardial infiltration. Thus, the degree of cardiac dysfunction may be related not only to the amount of amyloid deposited, but also to qualitative differences among fibrils.

Biohumoral markers as predictor of right ventricular dysfunction in AL Amyloidosis.

AIM: In AL amyloidosis, the importance of right ventricle (RV) involvement has recently been underlined and its role in predicting prognosis has been emphasized. Little is known about the relationship between RV involvement, N-terminal pro-brain natriuretic peptide (NT-proBNP) and troponin levels. Aim of our study was to clarify the relationship between NT-proBNP and troponin and RV involvement and analyze their independent value as predictors of RV dysfunction. METHODS AND RESULTS: We examined 76 consecutive patients with biopsy-proven AL amyloidosis. Each patient received complete clinical evaluation, troponin I, NT-proBNP assay and comprehensive echocardiographic evaluation. Considering a tricuspidal annulus plane systolic excursion (TAPSE) value <16 mm, 23 patients (30%) presented RV systolic dysfunction, whereas 53 (70%) did not. Patient with reduced TAPSE had thicker left ventricle (LV) walls and RV free walls, reduced LV fractional shortening, impaired LV diastolic function and worse LV and RV myocardial performance index. For RV dysfunction the best predictive value for NT-proBNP was identified as 2977 ng/l with sensitivity and specificity of 87% and 84%, respectively; best cut-off for troponin I was identified as 0.085 ng/l, with sensitivity and specificity of 85% and 90% respectively. At multivariable logistic regression analysis, both NT-proBNP and troponin I emerged as independent predictors of RV dysfunction presence but troponin appears to have a higher predictive power. CONCLUSION: Our study demonstrated that cut-off values of 2977 ng/ml for NT-proBNP and 0.085 ng/l for troponin were able to identify a subgroup of AL patients with RV dysfunction. Troponin I is more accurate and seems to be the best biohumoral marker of RV dysfunction.

A case of persistent hiccup after laparoscopic cholecystectomy.

A 79-year-old man, with history of recent laparoscopic cholecystectomy, came to our attention for persistent hiccup, dysphonia, and dysphagia. Noninvasive imaging studies showed a nodular lesion in the right hepatic lobe with transdiaphragmatic infiltration and increased tracer uptake on positron emission tomography. Suspecting a malignant lesion and given the difficulty of performing a percutaneous transthoracic biopsy, the patient underwent surgery. Histological analysis of surgical specimen showed biliary gallstones surrounded by exudative inflammation, resulting from gallbladder rupture and gallstones spillage as a complication of the previous surgical intervention. This case highlights the importance of considering such rare complication after laparoscopic cholecystectomy.

Cardiac amyloidosis: the heart of the matter.

Amyloidosis comprises a unique group of diseases that share in common the extracellular deposition of insoluble fibrillar proteins in organs and tissue including the heart. Cardiac amyloidosis could be primary a part of systemic acquired amyloidosis, or a result of heredofamilial amyloidosis. Although the infiltration of the heart from different types of amyloid results in restrictive cardiomyopathy that manifests with refractory congestive heart failure and conduction abnormalities, unequivocal identification of the deposited amyloidogenic protein is mandatory in order to avoid misdiagnosis and inappropriate treatment. Recent developments in imaging techniques and extracardiac tissue biopsy have minimized the need for invasive endomyocardial biopsy for amyloidosis. Despite advances in treatment, the prognosis of a patient with amyloidosis is still poor and depends upon the underlying disease, and the type and degree of dysfunction of the involved organs. Thus, early diagnosis is mandatory because patients with advanced disease are usually too ill for intensive therapy. This review outlines current approaches to diagnosis, assessment of disease severity, and treatment of cardiac amyloidosis.

[Severe recurrent intrahepatic cholestasis in systemic AL amyloidosis without obvious liver involvement: unexplained hepatic toxicity or a case of misdiagnosed liver amyloidosis?]. / Colestasi intraepatica in corso di amiloidosi sistemica AL senza evidenza istologica di un coinvolgimento epatico: un caso di epatopatia tossica o una mancata diagnosi di amiloidosi epatica?

We report the case of a 50-year-old woman who was admitted to the hospital for acute abdominal pain with nephrotic proteinuria, rapidly progressive renal failure, and moderate anemia. Laboratory tests showed mild Bence Jones (λ) proteinuria with negative serum immunofixation and a mild increase in λ free light chains. A bone marrow biopsy and a fat tissue aspirate showed multiple myeloma and amyloidosis. Because of the end-stage renal disease, the patient began regular dialysis treatment and was started on bortezomib 1.3 mg/m2 plus dexamethasone 40 mg on days 1, 4, 8 and 11 of 21-day cycles. Ten days later she complained of a new episode of abdominal pain with jaundice. A CT scan and an MRI scan ruled out all secondary causes of cholangitis including cancer. Acute intrahepatic cholestasis due to amyloid deposition was then hypothesized. After 4 well tolerated cycles of bortezomib and dexamethasone, blood tests showed a complete hematological response with full reversal of cholestasis. After three months, a new episode of abdominal pain occurred and this time the patient was operated on and found to have an intestinal volvulus. Because of the jaundice, a transjugular liver biopsy was performed showing no evidence of amyloid deposits. Two months later the patient died of septic shock. Although no autopsy was performed and the ultimate cause of the cholestasis could not be ascertained, amyloidosis remains the major culprit in this unfortunate case.