María Martino

Soft-tissue angiomatosis in adulthood: a case in the forearm showing a prominent myxoid adipose tissue component mimicking liposarcoma.

Soft‐tissue angiomatosis is a rare condition characterized by diffuse proliferation of benign vascular structures, accompanied by mature adipose tissue, which affect a large segment of the body in a contiguous fashion. Most patients present in childhood or adolescence. We report a case of large soft‐tissue angiomatosis, located in the left forearm of a 65‐year‐old man, which showed extensive intramuscular and intermuscular infiltration, and embracement of the radius and the main vascular and neural bundles of the forearm. Due to the deep location, large size, ill‐defined limits and non‐homogeneous composition with areas suggestive of an adipose component in the radiological examination, the lesion raised the possibility clinically of a liposarcoma. The myxoid adipose tissue component of the lesion, a feature previously undescribed, and the predominance of small, capillary‐sized vessels were considered concordant with the clinical diagnosis. This presentation is rare. The features of the lesion made complete surgical excision impossible and amputation of the extremity was considered the best treatment. The main differential diagnoses include infiltrating lipoma, myxolipoma, angiomyxolipoma, angiolipoma, intramuscular angioma, liposarcoma and low‐grade myxofibrosarcoma. In conclusion, angiomatosis might simulate liposarcoma, particularly if the mass is large, heterogeneous, myxoid and appears later in life.

Right Ventricular Structure and Function in Idiopathic Pulmonary Fibrosis with or without Pulmonary Hypertension.

To elucidate right ventricular (RV) function in patients with idiopathic pulmonary fibrosis (IPF) with and without pulmonary hypertension (PH) and its relation to other features of the disease.

Isolated non-compaction of the left ventricular myocardium in an adult treated with heart transplantation

Isolated left ventricular non‐compaction is a rare unclassified cardiomyopathy characterized by arrest of normal embryogenesis that results in the persistence of intertrabecular recesses and the development of a spongy myocardium. The pathological diagnosis is often first established at autopsy. Described herein is the case of a 57‐year‐old man with isolated non‐compaction of the left ventricle who had a 17 year history of worsening heart failure and was successfully treated with heart transplantation. To the authors’ knowledge only seven adult patients with non‐compaction cardiomyopathy have been reported to have undergone heart transplantation. Including the present case the mean age of the adult patients at transplantation was 39.5 years (range, 18–60 years). The male : female ratio was 3:1. Both ventricles were involved in 37.5% of cases. The mean follow up was 6.3 years (range, 2 months−17 years). One patient died because of a malignant tumor 9 years after transplantation. The morphological pattern of the isolated ventricular non‐compaction represents a pathological entity discernible from other cardiomyopathies and should be classified as a specific cardiomyopathy. It is likely that surgical pathologists will encounter this entity more frequently due to involvement in transplantation teams.

Prichard's structures of the fossa ovalis are age-related phenomena composed of nonreplicating endothelial cells: the cardiac equivalent of cutaneous senile angioma†

Prichards structures or minute endocardial deformities with lacunas of capillary size lined by plump endothelial cells located in the fossa ovalis are an age‐related alteration of unknown origin. In this report the histogenesis, the proliferative potential and the incidence in the aged of these structures are investigated. We have undertaken a prospective histological study of the fossa ovalis in a series of 111 consecutive hearts of patients aged ≥70 years obtained at autopsy. Included in this study was immunohistochemical staining for vimentin, CD31, CD34, thrombomodulin, c‐kit (CD117), Ki67 (MIB1) and vascular endothelial growth factor receptor 2 in six cases showing these structures. Prichards structures were observed in 50 hearts (45%), and were more frequent in males. We confirmed that these structures are age‐related phenomena. Subendothelial structures were more common than intracavitary structures. Most individuals had Prichards structures located on the right side; however, the structures were more numerous on the left side of the fossa ovalis. The immunohistochemical study revealed that Prichards structures consisted of adult, fully differentiated, postmitotic‐type endothelial cells. We suggest that Prichards structures are formed by infolding of the endothelial lining of the endocardium of the fossa ovalis as an irritational response to altered blood flow, eddies or turbulence. A parallel can be established between Prichards structures and senile angiomas as both structures increase with age; they are not genuine neoplasms; and they are overgrowths made up of endothelial cells with terminal differentiation. We propose that Prichards structures are the cardiac equivalent of cutaneous senile angioma.

Endobronchial ultrasound-guided transbronchial needle aspiration in the diagnosis of hilar and mediastinal lymph node metastases of melanoma

OBJECTIVE Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is a minimally invasive technique for investigating hilar and mediastinal lymphadenopathy. This study reports eleven cases in which EBUS-TBNA was used to assess mediastinal and hilar lymph nodes for the presence of metastatic melanoma. MATERIAL AND METHOD A retrospective study was performed of all patients who had a history of melanoma and underwent EBUS-TBNA to assess hilar or mediastinal lymphadenopathy for the presence of metastatic melanoma. In seven cases, molecular analysis to detect mutations in the BRAF gene was also used. RESULTS Eight patients had been diagnosed with malignant melanoma in the past (mean 54.4 months, range 18 to 115 months) while in the other three this tumor was primarily diagnosed in the staging phase. The male-female ratio was 6:5, and the mean age was 60.3 years (range 42 to 88 years). The mean hilar or mediastinal lymph node size detected with computed tomography was 3.0 cm (range 1.1 to 8.1 cm). Eight (72.7%) cases had metastases to the lung associated with metastases in the mediastinal lymph nodes. In four (50%) of these cases, the lung metastasis was solitary. Three (27.3%) cases had metastases in the mediastinal lymph nodes in absence of lung metastases. Metastatic melanoma was diagnosed by cytology and confirmed by cell block study with immunohistochemistry in all cases. BRAF mutations were detected in two (28.6%) of seven cases studied. CONCLUSION Cytology and tissue samples obtained from EBUS-TBNA are adequate to detect metastatic melanoma and permit in some cases the determination of biomarkers and identify the presence or absence of mutations in the BRAF gene. The procedure is safe, fast, and precise for the staging of melanoma.

Cholesterol granuloma in an antrochoanal polyp. A rare lesion in children

Antrochoanal polyps (ACPs) are an infrequent clinical entity. Cholesterol granulomas (CGs) are commonly associated with chronic middle ear disease but are rare in the paranasal sinuses. We describe a case of a 10-year-old girl with a concomitant CG in an ACP which was surgically excised by nasosinusal endoscopic surgery. To our knowledge, there are only five previously published cases of CGs in ACPs and of these, only two were pediatric cases. We describe a third case in the youngest patient yet reported. Increased intrasinus pressure may affect venous and lymphatic drainage, leading to hemorrhages with hemolysis and deposition of cholesterol crystals and their esters initiating the formation of granulomas in the polyp. In addition, the insufficient lymphatic drainage prevents the complete elimination of lipids, contributing to the formation of cholesterol granulomas. The treatment and the outcome of an ACP associated with a CG are the same as for usual ACPs.

Endobronchial ultrasound-guided transbronchial needle aspiration in the diagnosis of mediastinal metastases of clear cell renal cell carcinoma

Evaluation of mediastinal lymphadenopathy in patients with a previous diagnosis of renal cell carcinoma (RCC) is critical for the determination of further treatment. A minimally invasive method of cytology sampling of mediastinal lymph nodes using endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) has emerged as a useful tool in diagnosis. Between January 2010 and April 2018, we performed 1744 EBUS-TBNA studies of mediastinal and hilar lymph nodes for a variety of clinical indications including mediastinal malignancy. Sixteen patients (93.7% males, mean age 59.1 years, range 44-81 years) were diagnosed by cytological and cell block study to have metastatic clear cell RCC. Twelve patients had been diagnosed with clear cell RCC in the past (mean 39 months, range 4-89 months) while in four, the tumor was primarily diagnosed in the staging phase on the basis of EBUS-TBNA. The EBUS features of the mediastinal nodal masses included increase of size (mean 2.5 cm, range 1.6-3.8 cm), irregular, inhomogeneous, hypervascular, and hyperechoic echotexture. EBUS-TBNA is a procedure safe and effective for evaluating mediastinal lymphadenopathy in patients with clear cell RCC. Immunohistochemistry in the cell block is decisive for proper diagnosis. The cytologist plays a key role in the diagnosis of metastatic clear cell RCC due to the treatment implications that this neoplasm encompasses.

Supercritical Carbon Dioxide Extraction of Astaxanthin, Lutein, and Fatty Acids from Haematococcus pluvialis Microalgae

Haematococcus pluvialis microalgae in the red phase can produce significant amounts of astaxanthin, lutein, and fatty acids (FAs), which are valuable antioxidants in nutraceutics and cosmetics. Extraction of astaxanthin, lutein, and FAs from disrupted biomass of the H. pluvialis red phase using carbon dioxide (CO2) in supercritical fluid extraction (SFE) conditions was investigated using a bench-scale reactor in a semi-batch configuration. In particular, the effect of extraction time (20, 40, 60, 80, and 120 min), CO2 flow rate (3.62 and 14.48 g/min) temperature (50, 65, and 80 °C), and pressure (100, 400, and 550 bar.) was explored. The results show the maximum recovery of astaxanthin and lutein achieved were 98.6% and 52.3%, respectively, at 50 °C and 550 bars, while the maximum recovery of FAs attained was 93.2% at 65 °C and 550 bars.

Cellular myxoma of the vocal cord: a case report and review of the literature

Myxomas are rare in the vocal cords. A 69-year-old man was admitted with one-year history of progressive dysphonia. Laryngoscopy revealed a polypoid mass on the right vocal cord. The diagnosis was cellular myxoma. A review of the literature including the present case revealed eleven reported cases of myxoma. Ten cases were classic myxoma. To the best of our knowledge, cellular myxoma has not been previously reported in the vocal cord. Hypercellularity does not affect the behavior of cellular myxoma. However, its recognition is important to prevent confusion with the group of low-grade myxoid sarcomas. Cellular myxoma should be considered in the differential diagnosis of any vocal cord mass.

The clinical and biological response to allergen-specific immunotherapy with basophil activation test (BAT) in the atopic diseases

Background: Allergen-specific immunotherapy is an effective treatment and improves clinical symptoms of allergic asthma and rhinitis. Aim: To evaluate the response of basophilic activation in patients receiving subcoutaneous(SCIT) and sublingual(SLIT) immunotherapy. Methods: 28 patients (18M/10F; mean age 33.5; range 17- 46 years) with asthma and allergic rhinitis from polysensitisation to perennial allegens(Parietaria, grass pollen, Dermatophagoides) started treatment with allergen-specific immunotherapy(ITS). 23 patient received subcoutaneus therapy with modified allergens (allergoids)(19 for Parietaria, 4 for grass pollen)and 5 patients received SLIT.The SCIT was used before the pollination season.The BAT was performed at start-time, after 1 month, 6 months (during the pollen season) and after 12 months. Results: At T0, the mean levels of basophils activation (CD63+)were elevated(84% ± 10.2 in 19 allergic patients to Parietaria pollens, 64% ± 14.4 in 4 allergic pts to grass pollen, 5 pts 72 % ± 8.9 for DP/DF). At T1, the mean percentage of basophilic degranulation decreased (32% ± 8.2 for Parietaria, p <0.01; 26 % ± 8.4 for grass pollen)in patients treated with SCIT while in 5 patients treated with SLIT was not registered significant response (68 % ± 8.9 p= 1,3).Levels of basophilic activation were stable at T6 during the pollen season. At T 12, the levels of CD63+ decreased significantly in all patients regardless of the type of treatment SCIT or SLIT(p<0.05)in comparison to start time of treatment. Conclusion: The BAT has been validated in many IgE mediated disease and could have an important play to follow the favourable response to immunotherapy.