Marta Mayorga

Are there Predictive Factors of Severe Liver Fibrosis in Morbidly Obese Patients with Non-alcoholic Steatohepatitis?

Background: Non-alcoholic steatohepatitis (NASH) is a clinicopathological entity characterized by the presence of steatosis and lobular and/or portal inflammation with or without fibrosis. Patients with non-alcoholic fatty liver and fibrosis on liver biopsy have increased liver-related deaths. Methods: 181 wedge liver biopsies, taken at the time of bariatric surgery from patients with a mean body mass index (BMI) of 47, were studied. In all cases, the liver biopsy was performed without knowledge of the patients clinical and biochemical data, which were then examined with univariate and multivariate analysis. Results: Diagnosis of NASH was established in 105 patients (91%); 74 patients (70%) showed mild steatosis, 20 (19%) had moderate inflammation and fibrosis, and 11 (10%) had steatosis with severe fibrosis. None of the liver biopsies showed cirrhosis. Age was the only independent predictor of moderate and severe fibrosis (p=0.001). Conclusions: Since only age was a predictor of moderate or severe fibrosis, and no clinical or biochemical abnormalities detected slowly progressive hepatic fibrosis, liver biopsy is the only means of detecting progression to more advanced liver disease in a NASH patient.

Churg–Strauss syndrome and sudden cardiac death

Churg-Strauss syndrome is a rare disorder characterized by necrotizing vasculitis, granulomas with eosinophilic necrosis, and tissue infiltration by eosinophils. Sudden cardiac death is rarely described in Churg-Strauss syndrome. In this article, we describe a case of Churg-Strauss syndrome with multiorgan involvement manifested as sudden cardiac death. To the best of our knowledge, this form of presentation has not been reported. A 49-year-old woman was found dead in her room. No premonitory complaints had been noted during the days preceding her death. Past medical history did not reveal any relevant illness. At autopsy, multiorganic Churg-Strauss syndrome with prominent cardiac involvement was found. Therefore, this syndrome in the active vasculitic phase may be asymptomatic and may involve predominantly the heart. This variant of the syndrome may be fulminant and present as sudden cardiac death. This form can only be elucidated by autopsy study.

Increased expression of Ob‐Rb and its relationship with the overexpression of TGF‐β1 and the stage of fibrosis in patients with nonalcoholic steatohepatitis

Abstract: Aims: The main aim of this study was to examine the relationship of the leptin system in steatosis and nonalcoholic steatohepatitis (NASH). The study also analysed the pathogenic role of the leptin system in the development of hepatic fibrosis and its relation with the TGF‐β1 system.

Papillary (chromophil) renal cell carcinoma with mucinous secretion.

We investigated the presence of mucin in a series of 20 papillary renal cell carcinomas. Acid mucin was present in three cases (15%), in which basophilic mucin secretion was evident with hematoxylin-eosin. This secretion reacted positively with Mayers mucicarmine, Alcian blue and high-iron diamine, but was negative for PAS in all the cases, indicating the presence of sulphomucins. We describe two different types of mucin secretion: luminal and intracytoplasmic. The secretion was abundant, diffuse or extensive, luminal in two cases and intracellular in numerous scattered tumor cells in one case. All tumors were < 3 cm in diameter (low grade, stage I). In the three mucin-secreting papillary tumors mentioned above, the immunohistochemical and lectin studies indicate both a proximal and a distal tubular staining pattern. Mucinous secretion in these tumors can be ascribed either to modulation or direct metaplasia of the tumor epithelium. Mucin production, despite its low frequency, can be considered an additional feature of papillary renal cell carcinoma. Thus, the presence of luminal or intracytoplasmic mucin deposition does not exclude papillary renal cell carcinoma from the differential diagnosis in cases of intra- or extrarenal carcinomas.

PRIMARY AMYLOID TUMOR (AMYLOIDOMA) OF THE JEJUNUM WITH SPHEROID TYPE OF AMYLOID

The present report documents an incidentally discovered, solitary amyloidoma of the jejunum in a 70 year old man with no predisposing disorder or other sites of deposition of amyloid, as was demonstrated at autopsy. Amyloid tumors of the intestine have been reported only rarely. In those reports the deposits were conventional in type, forming large masses of acellular, eosinophilic, homogeneous material. In the case described here the deposits were in the form of corpora amylacea-like structures, or spheroids. To the best of our knowledge, the corpora amylacea-like structures, or spheroid amyloid, have not been previously described in bowel amyloidoma. This rare form of amyloid deposition should be recognized so as to prevent misdiagnosis.

Synchronous ipsilateral sebaceous lymphadenoma and acinic cell adenocarcinoma of the parotid gland

The synchronous or metachronous occurrence of 2 tumors of the salivary glands in a patient is rare. These tumors are usually benign and of the same histologic type. Here we report a 78-year-old woman who developed a sebaceous lymphadenoma and an acinic cell adenocarcinoma simultaneously in the left parotid gland. To our knowledge, this combination of neoplasms has not been reported before.

Glandular Inclusions in Inguinal Hernia Sacs: A Clinicopathological Study of Six Cases

Glandular inclusions in inguinal hernia sacs are not frequent. We present six cases of inguinal hernia with this finding, which represents an incidence of 2.6% in males and shows a predominance in the prepubertal stage. Five patients showed cryptorchidism and two cases were related to congenital malformations of the single umbilical artery type and 47,XY chromosome disorder with chromosomal marker. The most important differential diagnosis must be made with normal histological structures such as the vas deferens or epididymis. The mean diameter of the inclusions was 0.1988 mm and there was a significant difference in size between the inclusions and the vas deferens, but not the epididymis. Differentiation from the latter is based on the absence of a well-developed muscular coat in the wall of the inclusions. It is important to recognize that these inclusions can occur in hernia sacs because of the clinical and medicolegal implications that arise if they are confused with true epididymis or vas deferens. They may arise from paratesticular embryonal remnants.

Cardiac papillary fibroelastoma: Retrospective clinicopathologic study of 17 tumors with resection at a single institution and literature review

Cardiac papillary fibroelastomas (PFEs), which are mainly found in the valves, are rare benign tumors that can cause embolism. Single-center surgical experience in the treatment of this tumor is uncommon. All patients surgically treated for this neoplasm at our institution from January 1995 to October 2012 (15 patients with 17 lesions) were queried for clinical and pathologic characteristics, and the literature was reviewed. The mean age of detection was 55.8±11.48 years. Twenty percent of the patients were male. The tumor was an incidental finding in 60%. Symptoms directly related to PFEs occurred in 40% of patients. The most common clinical presentation in symptomatic cases was embolism (40%), mostly transient ischemic attack or stroke. Cardiac valves were predominantly involved (76.5%); the most commonly valve affected was the aortic valve (29.4%), followed by the mitral valve (17.6%). Concurrent valvular disease was observed in 41.7% of patients. The mean size of tumors was 11.4±7.9mm (range 2-25mm). In 86.7% of the patients, the tumor was solitary. The mitral valve was the most common origin of tumor systemic embolism. Fronds core could be the end stage of hypermature elastic fibers, which grows in apposition to young fibers. Simple surgical excision or valvular reconstruction was accomplished in most patients (58.3%). PFE is usually small, appears singly, and can cause potentially serious complications. Symptomatic and left side tumors should undergo surgical excision with valve-spare surgery when possible. Surgical removal of PFE is safe, efficacious, and definitive.

Myeloid sarcoma of the extrahepatic bile ducts presenting as obstructive jaundice.

We report a rare case of myeloid sarcoma (MS) of the extrahepatic bile ducts presenting as obstructive jaundice in a patient without leukemia at time of diagnosis. A 75‐year‐old female presented with a one‐month history of abdominal pain and jaundice. Computerized tomography scan of the abdomen showed stenosis of the extrahepatic bile ducts. Endoscopic retrograde cholangiography disclosed an irregular narrowing of the common biliary duct, suggestive of a cholangiocarcinoma, and resection was performed. Histologic examination showed diffuse transmural infiltration of malignant cells. These cells exhibited medium‐sized round nuclei with central nucleoli and eosinophilic cytoplasm, and were strongly positive for myeloperoxidase, CD68, lysozyme, CD45, CD117 (c‐kit protein) and CD43. Eight months following surgery the patient presented with multiple cutaneous nodules and bone marrow trephine biopsy showed acute myelomonocytic leukemia. A literature search identified two previously reported cases of MS of the extrahepatic biliary duct. MS should be taken into consideration in the differential diagnosis of a patient with obstructive jaundice. Immunohistochemistry is essential for a correct diagnosis.

Dermatofibrosarcoma protuberans of the vulva: clinico‐ pathological, immunohistochemical and flow cytometric study of a case

Vulvar niesenchyrnal tumors are rare. Sarcomas account for only three per cent of vulvar tumors. Leiomyosarcoma is the most common, followed by malignant fibrous histiocytoma ( 1). Dermatofibrosarcoma protuberans, which is considered a low-grade sarcoma, is an uncommon cutaneous neoplasm characterized by local aggressiveness, a marked tendency for recurrence and an extremely low metastatic potential. DP most often involves the trunk and proximal extremities. To our knom4edge. only eleven cases of DP of the \.ul\.a have been reported previously ( 1-9). We present an additional case of a tumor arising at this unusual site. and report the findings of the immiiiiohistocheniical and flow cytometric studies.