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1.
Acta Vet Scand ; 63(1): 45, 2021 Nov 22.
Artigo em Inglês | MEDLINE | ID: mdl-34809688

RESUMO

BACKGROUND: Primary laryngeal neoplasms are rare in cats, with lymphoma and squamous cell carcinoma being the most commonly diagnosed tumour types. These tumours are usually highly aggressive, difficult to treat, and have a poor prognosis. Here an undifferentiated laryngeal carcinoma with hyaline bodies in a cat is reported. CASE PRESENTATION: A 13-year-old cat was presented for progressive respiratory signs. Diagnostic procedures revealed a partially obstructive laryngeal mass. Cytology was compatible with a poorly differentiated malignant tumour, with neoplastic cells frequently containing large intracytoplasmic hyaline bodies. After 1 month the patient was euthanised due to a worsening clinical condition and submitted for post-mortem examination, which confirmed the presence of two laryngeal masses. Histopathology confirmed the presence of an undifferentiated neoplasm with marked features of malignancy. Strong immunolabelling for pancytokeratin led to a diagnosis of undifferentiated carcinoma, however, histochemical and immunohistochemical investigations could not elucidate the origin of the large intracytoplasmic hyaline bodies observed in tumour cells, which appeared as non-membrane bound deposits of electron-dense material on transmission electron microscopy. CONCLUSION: This is the first report of primary undifferentiated laryngeal carcinoma in a cat. Our case confirms the clinical features and the short survival that have been reported in other studies describing feline laryngeal tumours. Moreover, for the first time in feline literature, we describe the presence of intracytoplasmic hyaline bodies in neoplastic cells that were compatible with the so-called hyaline granules reported in different human cancers and also in the dog.


Assuntos
Carcinoma , Doenças do Gato , Neoplasias Laríngeas , Laringe , Animais , Carcinoma/diagnóstico , Carcinoma/veterinária , Doenças do Gato/diagnóstico , Gatos , Hialina , Neoplasias Laríngeas/diagnóstico , Neoplasias Laríngeas/veterinária , Microscopia Eletrônica de Transmissão/veterinária
2.
An Bras Dermatol ; 96(6): 730-734, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34544637

RESUMO

Lipoid proteinosis is a rare autosomal recessive disease, characterized by hyaline deposits of PAS-positive material in tissues due to mutations in the ECM1 gene. This study evaluated the ultrastructure of the skin of a 6-year-old child affected by this condition. The light microscopy identified PAS-positive hyaline deposits, which were more intense in the papillary dermis. Scanning electron microscopy of the dermis showed a compact papillary dermis and fibrillar deposits in the middle dermis. Transmission electron microscopy clearly showed the deposition of fibrillar material in the dermis, forming clusters adherent to elastic fibers, between the collagen bundles and the collagen fibers, and also filling up the cytoplasm of dermal fibroblasts.


Assuntos
Proteinose Lipoide de Urbach e Wiethe , Criança , Colágeno , Proteínas da Matriz Extracelular , Humanos , Hialina , Microscopia , Pele
3.
Biofabrication ; 13(4)2021 08 31.
Artigo em Inglês | MEDLINE | ID: mdl-34404032

RESUMO

The pellet formation has been regarded as a golden standard forin vitrochondrogenic differentiation. However, a spatially inhomogeneous chondrogenic microenvironment around a pellet resulted from the use of a traditional impermeable narrow tube, such as the conical tube, undermines the differentiation performance and therapeutic potential of differentiated cartilage pellet in defective articular cartilage treatment. To address this drawback, a perichondrium-inspired permeable nanofibrous tube (PINaT) well with a nanofibrous wall permeable to gas and soluble molecules is proposed. The PINaT well was fabricated with a micro deep drawing process where a flat thin nanofibrous membrane was transformed to a 3.5 mm deep tube well with a ∼50µm thick nanofibrous wall. Similar toin vivoperichondrium, the PINaT well was found to allow oxygen and growth factor diffusion required for chondrogenic differentiation across the entire nanofibrous wall. Analyses of gene expressions (COL2A1, COL10A1, ACAN, and SOX9), proteins (type II and X collagen), and glycosaminoglycans contents were conducted to assess the differentiation performance and clinical efficacy of differentiated cartilage pellet. The regulated spatially homogeneous chondrogenic microenvironment around the human induced pluripotent stem cell-derived pellet (3 × 105cells per pellet) in the PINaT well remarkably improved the quality of the differentiated pellet toward a more hyaline-like cartilage pellet. Furthermore, an accelerated chondrogenic differentiation process of the pellet produced by the PINaT well was achieved for 14 days, demonstrating a hyaline cartilage-specific marker similar to the control pellet differentiated for 20 days. Finally, the enhanced clinical efficacy of the hyaline-like cartilage pellet was confirmed using an osteochondral defect rat model, with the repaired tissue resembling hyaline cartilage rather than fibrous cartilage after 8 weeks of regeneration.


Assuntos
Células-Tronco Pluripotentes Induzidas , Nanofibras , Animais , Cartilagem Articular , Diferenciação Celular , Condrócitos , Condrogênese , Humanos , Hialina , Cartilagem Hialina , Ratos
4.
J Eur Acad Dermatol Venereol ; 35(11): 2308-2316, 2021 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-34331822

RESUMO

BACKGROUND: Arteriolosclerotic ulcers of Martorell are histologically characterized by hyaline arteriolosclerosis resulting in concentric occlusion of the arteriolar lumina. Although several authors have previously reported on hyaline changes in hypertensive arteriolopathies, so far, little information is available on the molecular composition of hyaline wall depositions. OBJECTIVES: This study aimed at the molecular characterization of hyaline arteriolar deposits in patients with hypertensive arteriolopathy using confocal Raman spectroscopy. METHODS: Samples of patients diagnosed with arteriolosclerotic ulcers of Martorell were analysed using confocal Raman spectroscopy. The findings were correlated with histological analyses. Skin samples from healthy, non-hypertensive patients served as controls. RESULTS: Confocal Raman spectroscopy analysis revealed that subendothelial hyaline deposits in arteriolosclerotic ulcers are mainly composed of collagen and phospholipids, in particular phosphatidylcholine. The presence of collagen within hyaline deposits was confirmed by Masson's Trichrome and Picrosirius Red staining. Additionally, the presence of collagen could also be shown for hypertensive nephrosclerosis. Actin was markedly decreased in hyalinized compared to control vessels, corresponding to the loss of smooth muscle cells in the process of hyalinization. This was confirmed by immunofluorescence staining for α-smooth muscle actin and desmin. CONCLUSION: The present findings suggest that arteriolar hyaline deposits in hypertensive arteriolopathy are mainly composed of collagen and phospholipids, in particular phosphatidylcholine. Together with the concurrent absence of actin, these findings suggest that potentially critical disease mechanisms involve pressure-induced vascular smooth muscle cell apoptosis with subsequent deposition of collagen.


Assuntos
Arteriolosclerose/complicações , Colágeno/análise , Hialina/química , Hipertensão/complicações , Úlcera da Perna/etiologia , Fosfolipídeos/análise , Humanos , Análise Espectral Raman
5.
Bull Tokyo Dent Coll ; 62(2): 89-98, 2021 Jun 04.
Artigo em Inglês | MEDLINE | ID: mdl-33994426

RESUMO

Capnocytophaga ochracea possesses a type-IX secretion system that exports proteins which have a gliding motility-associated C-terminal (CTD) domain. This system is found in several species of the Bacteroidetes phylum. Hyalin, a large protein encoded by Coch_0033 in C. ochracea ATCC 27872, has a CTD domain and is posited to be involved in quorum sensing according to the database of the Kyoto Encyclopedia of Genes and Genomes. This suggests that it plays a role in biofilm formation via interbacterial communication. The aim of this study was to investigate the potential role of the hyalin-like protein coded by the Coch_0033 gene in gliding and biofilm formation of C. ochracea. A hyalin-like protein-deficient mutant strain of C. ochracea, designated mutant WR-1, was constructed through insertion of the ermF-ermAM cassette into the target gene. The spreading feature at the edge of the colony was lost in the mutant strain. Crystal violet and confocal laser scanning microscopy revealed no difference between the quantity of biofilm organized by the mutant and that organized by the wild-type strain. These data suggest that the hyalin-like protein encoded by the Coch_0033 gene is indeed involved in C. ochracea gliding activity.


Assuntos
Capnocytophaga , Hialina , Proteínas de Bactérias/genética , Bacteroidetes/genética , Biofilmes , Capnocytophaga/genética
6.
Dentomaxillofac Radiol ; 50(7): 20200603, 2021 Oct 01.
Artigo em Inglês | MEDLINE | ID: mdl-33877880

RESUMO

OBJECTIVES: Salivary duct carcinoma is an aggressive tumour commonly showing local invasion and/or nerve palsy. However, their CT/MRI findings, especially, regarding T2WI, and the diffusion-weighted-image (DWI), were not well known. In this study, we correlated the CT/MRI appearance and the pathological findings containing the nerve invasion cases such as a facial nerve. METHODS: We reviewed 14 cases of SDC (parotid = 11, submandibular = 2, minor salivary gland = 1) pathologically proven peripheral nerve involvement. Their CT findings of all patient including dynamic contrast-enhancement study、MRI (n = 9) and DWI (n = 6) were also analyzed with histopathological correlation. RESULTS: On contrast-enhanced CT, the solid component was moderately enhanced. On MRI, T2WI central low signal core (n = 6) with peripheral high intensity rim (n = 5) was frequently observed except heterogeneous low and high (n = 1), diffuse low (n = 1), and high (n = 1) signal cases. The hyaline degenerative area located in the tumour core was poorly enhanced. Eleven tumours had an ill-defined margin, reflecting invasive tumour growth. On DWI, they showed high signal [the central low and peripherally high (n = 4), and diffuse (n = 1), heterogeneously high signal (n = 1)]. The mean ADC value was 1.148 ~ 0.961 x 10-3 mm2/s. With pathological correlation, the central low signal area on T2WI reflected hyaline degeneration. The sites of gross nerve involvement were revealed as tubular or branching structures on CE-CT (n = 3), and MRI (n = 1). CONCLUSIONS: (1) We frequently observed a central low signal area on T2WI/DWI in SDC. With histopathological correlation, it corresponded to the central hyaline degeneration with the peripheral viable tumour. 2) The gross nerve involvement might be detected as a strongly enhancement structure.


Assuntos
Carcinoma , Ductos Salivares , Imagem de Difusão por Ressonância Magnética , Humanos , Hialina , Imageamento por Ressonância Magnética , Nervos Periféricos , Estudos Retrospectivos , Ductos Salivares/diagnóstico por imagem , Tomografia Computadorizada por Raios X
7.
Acta Biomater ; 128: 236-249, 2021 07 01.
Artigo em Inglês | MEDLINE | ID: mdl-33894352

RESUMO

Regenerative therapies for articular cartilage are currently clinically available. However, they are associated with several drawbacks that require resolution. Optimizing chondrocyte expansion and their assembly, can reduce the time and costs of these therapies and more importantly increase their clinical success. In this study, cartilage organoids were quickly mass produced from bovine chondrocytes with a new suspension expansion protocol. This new approach led to massive cell proliferation, high viability and the self-assembly of organoids. These organoids were composed of collagen type II, type VI, glycosaminoglycans, with Sox9 positive cells, embedded in a pericellular and interterritorial matrix similarly to hyaline cartilage. With the goal of producing large scale tissues, we then encapsulated these organoids into alginate hydrogels with different viscoelastic properties. Elastic hydrogels constrained the growth and fusion of the organoids inhibiting the formation of a tissue. In contrast, viscoelastic hydrogels allowed the growth and fusion of the organoids into a homogenous tissue that was rich in collagen type II and glycosaminoglycans. The encapsulation of organoids to produce in vitro neocartilage also proved to be superior to the conventional method of encapsulating 2D expanded chondrocytes. This study describes a multimodal approach that involves chondrocyte expansion, organoid formation and their assembly into neohyaline-cartilage which proved to be superior to the current standard approaches used in cartilage tissue engineering. STATEMENT OF SIGNIFICANCE: In this manuscript, we describe a new and simple methodology to quickly mass produce self-assembling cartilage organoids. Due to their matrix content and structure similarities with native cartilage, these organoids on their own have the potential to revolutionize cartilage research and the manner in which we study signaling pathways, disease progression, tissue engineering, drug development, etc. Furthermore, these organoids and their fast mass production were combined with a key relatively ignored hydrogel characteristic, viscoelasticity, to demonstrate their fusion into a neo-tissue. This has the potential to open the door for large scale cartilage regeneration such as for entire joint surfaces.


Assuntos
Cartilagem Articular , Hidrogéis , Animais , Bovinos , Condrócitos , Hialina , Cartilagem Hialina , Organoides , Engenharia Tecidual
8.
Rom J Intern Med ; 59(2): 127-133, 2021 Jun 01.
Artigo em Inglês | MEDLINE | ID: mdl-33565308

RESUMO

Background. Few data with adequate evidence exists as regards the effect of Cyclosporine (CsA) and mycophenolate mofetil (MMF) on pathological prognostic parameters in patients with steroid resistant focal segmental glomerulosclerosis (FSGS). The purpose of the present study is to compare the effect of cyclosporin and mycophenolate mofetil in addition to steroids on functional and histopathologic renal parameters in patients with steroid resistant FSGS one year after treatment.Material and methods. Thirty-seven adults with primary FSGS patients resistant to steroid therapy consecutively randomized to treatment with either MMF or cyclosporine. Low dose prednisolone added to both groups. Glomerular filtration rate (GFR) and blood pressure (BP) were determined at all examinations and a second renal biopsy was taken 12 months after treatment with either of cyclosporin and mycophenolate mofetil.Results. GFR significantly increased in MMF group p < 0.01 after 6 months and unchanged after 12 months. On the other hand, GFR significantly decrease in CsA group p < 0.001 after 6 months and reduced more after 12 months p < 0.001 compared to base line levels. There was a significant difference of GFR between the 2 groups at 6 months p < 0.001. The extent of proteinuria decreased significantly in CsA group after 12 months p < 0.001. The extent of arteriolar hyalinosis increased significantly in CsA group (0.78 to 1.81 score, p < 0.001) but was unchanged in MMF group (0.93 to 0.96 score), whereas interstitial fibrosis increased to same level in both groups (grade 3).Conclusion. Conversion to MMF in those patients may be superior to CsA as regards GFR after 12 months after treatment in spite of the presence of greater level of protein excretion. The increased arteriolar hyalinosis during CsA treatment most likely results in higher BP compared to MMF treatment in patients with FSGS resistant to steroids.


Assuntos
Ciclosporina/uso terapêutico , Glomerulosclerose Segmentar e Focal/tratamento farmacológico , Glomerulosclerose Segmentar e Focal/fisiopatologia , Imunossupressores/uso terapêutico , Ácido Micofenólico/uso terapêutico , Adulto , Arteríolas/fisiopatologia , Pressão Sanguínea/efeitos dos fármacos , Diarreia/fisiopatologia , Resistência a Medicamentos , Quimioterapia Combinada , Oftalmopatias Hereditárias/fisiopatologia , Feminino , Fibrose/patologia , Taxa de Filtração Glomerular/efeitos dos fármacos , Glomerulosclerose Segmentar e Focal/metabolismo , Glomerulosclerose Segmentar e Focal/patologia , Glucocorticoides/uso terapêutico , Humanos , Hialina/metabolismo , Enteropatias/fisiopatologia , Masculino , Prednisolona/uso terapêutico , Estudos Prospectivos , Proteinúria , Anormalidades da Pele/fisiopatologia , Resultado do Tratamento , Doenças Vasculares/fisiopatologia
9.
Ann Diagn Pathol ; 51: 151706, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33516059

RESUMO

Two cases of primary intrapulmonary hyalinizing spindle cell tumor with giant rosettes are presented. The patients are one woman and one man ages 37 and 42 years respectively. Both patients presented with non-specific symptoms of cough, dyspnea, and chest pain. Imaging revealed the presence of an intrapulmonary mass. One tumor was located in the left lower lobe while the other tumor was in the right upper lobe. Both patients underwent lobectomy. The tumors ranged from 2.4 to 3.0 cm in greatest dimension and were characterized by the presence of a bland spindle cell proliferation with areas of hyalinization and the presence of the so-called giant rosettes. Immunohistochemical stains were performed and the spindle cell component show positive staining for vimentin and negative staining for Bcl-2, CD34, STAT6, p40. Keratin immunohistochemical stain highlighted the entrapped alveolar epithelium while S-100 protein showed weak focal staining in the spindle cells. Both patients have remained alive and well without evidence of recurrence or metastasis for a period of 6 to 14 months post-surgical resection. The cases herein presented highlight the ubiquitous distribution of this tumor and underscores the importance of keeping this particular tumor in the differential diagnosis of spindle cell tumors of the lung.


Assuntos
Fibroma/diagnóstico , Hialina/metabolismo , Neoplasias Pulmonares/patologia , Sarcoma/diagnóstico , Adulto , Proliferação de Células , Dor no Peito/diagnóstico , Dor no Peito/etiologia , Tosse/diagnóstico , Tosse/etiologia , Diagnóstico Diferencial , Dispneia/diagnóstico , Dispneia/etiologia , Feminino , Fibroma/metabolismo , Fibroma/patologia , Fibroma/cirurgia , Humanos , Hialina/ultraestrutura , Imuno-Histoquímica/métodos , Neoplasias Pulmonares/cirurgia , Masculino , Proteínas S100/metabolismo , Sarcoma/metabolismo , Sarcoma/patologia , Sarcoma/cirurgia , Neoplasias de Tecidos Moles/patologia , Neoplasias de Tecidos Moles/cirurgia , Resultado do Tratamento , Vimentina/metabolismo
10.
J Cutan Pathol ; 48(5): 701-705, 2021 May.
Artigo em Inglês | MEDLINE | ID: mdl-33469928

RESUMO

Castleman disease (CD) is an unusual lymphoproliferative disorder characterized by multiple lymphadenopathy accompanied by marked systemic inflammatory symptoms. CD can be unicentric (UCD) or multicentric (MCD), and it can be classified into three types based on histopathology: hyaline vascular type, plasma cell type, and mixed hyaline vascular and plasma cell type. CD involving skin is an unusual clinical manifestation. Abnormalities including rash, hyperpigmentation, cherry hemangiomatosis, paraneoplastic pemphigus, and Kaposi sarcoma have been reported to occur in MCD. Here, we reported an unusual case of MCD which presented initially with disseminated dark brown papules, patches, and plaques, and pathologically demonstrated plasma cell type CD, a finding which is rarely reported. The peculiar clinicopathological features will be discussed.


Assuntos
Medula Óssea/patologia , Hiperplasia do Linfonodo Gigante/diagnóstico , Linfonodos/patologia , Transtornos Linfoproliferativos/patologia , Plasmócitos/patologia , Adulto , Anti-Infecciosos Locais/administração & dosagem , Anti-Infecciosos Locais/uso terapêutico , Anti-Inflamatórios não Esteroides/administração & dosagem , Anti-Inflamatórios não Esteroides/uso terapêutico , Biópsia , Hiperplasia do Linfonodo Gigante/tratamento farmacológico , Hiperplasia do Linfonodo Gigante/patologia , Quimioterapia Combinada , Feminino , Glucocorticoides/administração & dosagem , Glucocorticoides/uso terapêutico , Hemangioma/patologia , Humanos , Hialina , Hiperpigmentação/patologia , Imunossupressores/administração & dosagem , Imunossupressores/uso terapêutico , Anormalidades da Pele/patologia , Resultado do Tratamento
11.
Oral Dis ; 27(3): 391-403, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31981280

RESUMO

Hyaline ring granuloma (HRG) is a rare and atypical foreign-body granulomatous chronic inflammatory condition. Our aim was to make a critical review of the literature cases and also to illustrate histochemical and immunohistochemical findings of a new case. A modified PICO strategy was used. Inclusion criteria were met by case reports and case series with a histologically confirmed diagnosis of HRG, necessarily in mouth or maxillary bones. Exclusion criteria were no access to the full-text paper. The search was done up to 2019, totalizing 231 selected cases in 54 papers. The new case was analyzed through PAS, Azan-Mallory's trichrome, and CD34 and CD68 reactions. HRG is found mainly in the mandible of men, edentulous and prosthesis users, between 51 and 60 years of age, with 0.5-120 months of duration, usually causing edema and pain or discomfort. Endogenous and exogenous theories may be complementary in HRG. Eosinophilic areas could represent particles of vegetal origin, while the hyaline material could be the result of collagen degradation. Considering the material is not always present or even is ring-shaped, we suggest the change from "hyaline ring granuloma" to "granuloma induced by vegetal inoculation."


Assuntos
Granuloma de Corpo Estranho , Hialina , Criança , Pré-Escolar , Odontólogos , Face , Humanos , Lactente , Recém-Nascido , Masculino , Boca
12.
Clin Imaging ; 70: 51-55, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-33120290

RESUMO

Hyaline fibromatosis syndrome (HFS) is a rare, progressive, autosomal recessive disorder that presents with connective tissue deposition of amorphous hyaline material within the musculocutaneous tissue and/or visceral organs. HFS presents clinically in infancy or early childhood and can result in severe disability and life threatening complications. Given the rarity of the disorder, the imaging characteristics of HFS are seldom described in the literature. We describe a case of a 25-year-old patient presenting with bilateral knee pain, limited range of motion in her extremities, and lower extremity weakness with detailed MR imaging demonstrating the first case of multifocal intra-articular deposition of hyaline material within several joints.


Assuntos
Fibroma , Hialinose Sistêmica , Adulto , Criança , Pré-Escolar , Feminino , Humanos , Hialina , Hialinose Sistêmica/diagnóstico por imagem , Articulação do Joelho , Imageamento por Ressonância Magnética , Dor
13.
Histopathology ; 78(7): 987-999, 2021 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-33249657

RESUMO

AIMS: In the evolving era of precision medicine, increasing emphasis is placed on detecting molecular alterations driving the development of specific cancers and targeting them with matched therapies that can yield the best outcomes for patients. Lung adenocarcinomas with uncommon actionable alterations, including MET exon 14 skipping (METex14), ERBB2 and BRAF mutations, are rare and poorly characterised cancers. METHODS AND RESULTS: To more clearly understand the histopathological features of lung adenocarcinoma with uncommon actionable alterations, we compared the histological features of 678 cases with mitogenic driver alterations from 996 surgically resected lung adenocarcinomas. Genomic data from our cohort revealed METex14, ERBB2 and BRAF mutations in 13, 16 and 15 cases, respectively. Patients who had lung adenocarcinoma with METex14 were often elderly females. Histological features such as clear cell features (23%), hyaline globules (31%) and nuclear pleomorphism (39%) were the most frequently identified in METex14-positive cases; among those, three cases (23%) had tumour cells with bizarre giant or multilobulated nuclei. Additionally, the micropapillary pattern was the most frequently identified in ERBB2-mutated lung adenocarcinoma (31%). Lung adenocarcinoma with BRAF mutations tended to be less invasive, and the BRAF V600E mutation was identified in only one case with lepidic adenocarcinoma. Immunohistochemically, all METex14, ERBB2 and BRAF-positive tumours, except for invasive mucinous adenocarcinoma, were positive for thyroid transcription factor 1 (TTF-1). CONCLUSIONS: Our data from Japanese patients showed that lung adenocarcinoma with METex14 had unique clinicopathological characteristics: tumour cells with marked nuclear pleomorphism, hyaline globules and expression of TTF-1 in elderly women who never or lightly smoked.


Assuntos
Adenocarcinoma de Pulmão , Proteínas Proto-Oncogênicas c-met/genética , Fator Nuclear 1 de Tireoide/metabolismo , Adenocarcinoma de Pulmão/genética , Adenocarcinoma de Pulmão/patologia , Idoso , Idoso de 80 Anos ou mais , Estudos de Coortes , Genômica , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Hialina/citologia , Neoplasias Pulmonares/patologia , Pessoa de Meia-Idade , Mutação , Proteínas Proto-Oncogênicas c-met/metabolismo , Receptor ErbB-2/genética , Receptor ErbB-2/metabolismo , Fator Nuclear 1 de Tireoide/genética
14.
J Cutan Pathol ; 48(7): 896-901, 2021 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-33241586

RESUMO

Recently, a novel group of CD34+ and S100+ spindle cell tumors with distinctive stromal and perivascular hyalinization showing recurrent gene fusions involving RAF1, BRAF, NTRK1/2/3, and RET has been identified. ALK rearrangements have been rarely reported in this group of tumors. We report a 24-year-old woman with a 1.5-cm pink mass of the scalp. The tumor was made of spindle cells organized in fascicles or haphazardly arranged in a patternless architecture, with areas of stromal and perivascular hyalinization. The tumor cells diffusely expressed CD34 and S100, without SOX-10 expression. The tumor showed diffuse immunopositivity for ALK. RNA sequencing using next-generation sequencing (NGS) detected an EML4-ALK fusion. This case extends the spectrum of this newly described group of CD34+/S100+ spindle cell tumors at the molecular-genetic level. Dermatopathologists should be aware of this recent entity, as it may fall in the differential diagnosis of many other spindle cell tumors with CD34 expression. NGS-based techniques should be performed when facing spindle cell tumors with similar morphology and immunophenotype. Identification of kinase fusions is essential for the precise classification and better knowledge of these tumors, and for targeted therapy in rare aggressive cases.


Assuntos
Antígenos CD34/metabolismo , Proteínas S100/metabolismo , Neoplasias de Tecidos Moles/metabolismo , Neoplasias de Tecidos Moles/patologia , Vasos Sanguíneos/metabolismo , Vasos Sanguíneos/patologia , Diagnóstico Diferencial , Feminino , Sequenciamento de Nucleotídeos em Larga Escala/métodos , Humanos , Hialina/metabolismo , Imuno-Histoquímica/métodos , Proteínas de Fusão Oncogênica , Análise de Sequência de RNA/métodos , Neoplasias de Tecidos Moles/diagnóstico , Células Estromais/metabolismo , Células Estromais/patologia , Recusa do Paciente ao Tratamento , Adulto Jovem
15.
Gan To Kagaku Ryoho ; 48(13): 2058-2060, 2021 Dec.
Artigo em Japonês | MEDLINE | ID: mdl-35045492

RESUMO

The patient was a 29-year-old female with a chief complaint of transient right-sided abdominal pain. A CT scan revealed homogenously contrasted tumor of 4 cm in diameter with smooth margins and clear borders in the lower part of the pancreatic head. The tumor was contrast-enhanced on MRI and stained on abdominal angiography using the proximal branch of the right colonic artery as a feeding vessel. PET scan showed moderate accumulation. Malignancy could not be ruled out, and tumor resection including the ascending colon was performed. The pathological diagnosis was hyaline vascular-type Castleman's disease in the mesentery of the colon.


Assuntos
Hiperplasia do Linfonodo Gigante , Adulto , Angiografia , Hiperplasia do Linfonodo Gigante/diagnóstico por imagem , Hiperplasia do Linfonodo Gigante/cirurgia , Feminino , Humanos , Hialina , Mesentério , Tomografia Computadorizada por Raios X
17.
Ann Diagn Pathol ; 49: 151639, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-33069084

RESUMO

Hyalinized stroma (HS) is a dense, eosinophilic, and amorphous extracellular material in the stroma. HS is observed in several tumors; however, it has not been comprehensively studied in pancreatic intraductal papillary mucinous neoplasm (IPMN) or intraductal oncocytic papillary neoplasm (IOPN). Here, we aimed to evaluate the immunohistochemical and microscopic characteristics of HS in IPMN and IOPN. The prevalence of HS was determined in 168 cases of IPMN, including intestinal type (IPMN-I), gastric type (IPMN-G), and pancreatobiliary type (IPMN-PB), as well as in 11 cases of IOPN. Immunohistochemical staining for laminin and collagen (types I, II, III, IV, and V), as well as Congo red staining were performed in IPMN and IOPN cases containing HS. The prevalence of HS among the IPMN and IOPN specimens was 1.2% (2/168 cases) and 45.5% (5/11 cases), respectively. The prevalence rates of HS in each IPMN subtype were as follows: 2.2% (2/91 cases) in IPMN-G, and 0% in IPMN-PB and IPMN-I. All seven HS cases were positive for collagen I, III, IV, and V but were negative for Congo red staining. Most cases showed negative, focal, or weak expression of laminin and type II collagen. These findings indicate that HS is associated with IOPN and is primarily composed of collagen fibers.


Assuntos
Adenoma Oxífilo/patologia , Hialina , Neoplasias Intraductais Pancreáticas/patologia , Microambiente Tumoral , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Imuno-Histoquímica , Masculino , Pessoa de Meia-Idade
18.
Am J Clin Pathol ; 154(6): 859-866, 2020 11 04.
Artigo em Inglês | MEDLINE | ID: mdl-32880623

RESUMO

OBJECTIVES: Soft tissue sarcomas are a group of tumors derived from the mesenchymal origin. Historically, they have been classified according to morphologic and immunohistochemical characteristics. The advent of multiplexed next-generation sequencing (NGS), specifically RNA sequencing, has modified the classification of such tumors and others by determining categorization based on molecular alterations. The NUTM1 rearrangement, previously thought to be present only in carcinomas, has recently been reported in poorly differentiated high-grade sarcomas of the soft tissue. We present the first reported case of an epithelioid hyalinizing sarcoma harboring the MGA-NUTM1 fusion in an acral site. METHODS: Histopathologic, immunohistochemical, and molecular testing were performed on resection tissue. RESULTS: Histologically, the tumor showed an epithelioid morphology with prominent background hyalinization. Immunohistochemically, the tumor expressed CD99 and nuclear NUT-1. By NGS the tumor harbors MGA-NUTM1 fusion. CONCLUSIONS: Our findings support more extensive use of NGS for accurate sarcoma classification and identification of potential therapeutic targets. Furthermore, they corroborate the fact that NUTM1-rearranged soft tissue tumors represent a spectrum of heterogeneous morphologic entities. This case also highlights the utility of NUT-1 immunohistochemical study as a possible screening tool for NUTM1-fused sarcomas.


Assuntos
Fatores de Transcrição Hélice-Alça-Hélice Básicos/genética , Proteínas de Neoplasias/genética , Proteínas Nucleares/genética , Sarcoma/genética , Sarcoma/patologia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Ciclofosfamida/uso terapêutico , Dactinomicina/uso terapêutico , Etoposídeo/administração & dosagem , Etoposídeo/análogos & derivados , , Ossos do Pé/patologia , Fusão Gênica , Humanos , Hialina , Ifosfamida/administração & dosagem , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Músculo Esquelético/patologia , Metástase Neoplásica/tratamento farmacológico , Metástase Neoplásica/patologia , Recidiva Local de Neoplasia/patologia , Compostos Organofosforados/administração & dosagem , Sarcoma/cirurgia , Vincristina/uso terapêutico
19.
Rev Esp Patol ; 53(3): 182-187, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32650969
20.
Cornea ; 39(7): 868-876, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32398423

RESUMO

PURPOSE: To report rare dematiaceous and hyaline fungal pathogens causing fungal keratitis (FK) at our center and review the published literature in this field. METHODS: In a retrospective review of FK cases from 2005 to 2011, a total of 14 rare pathogens causing isolated cases of FK of a total of 393 isolates were revived from our collection and reconfirmed by using molecular techniques. The in vitro antifungal susceptibility testing (AFST) was performed against a 6-antifungal drug panel by the CLSI microbroth-dilution method. We reviewed the literature on keratitis due to these rare fungi. RESULTS: Two novel fungi, Alternaria tenuissima and Epicoccum nigrum, were reported in addition to 6 dematiaceous (black pigmented) fungi (Acrophialophora fusispora, Chaetomium globosum, Cladophialophora carionii, Nigrospora sphaerica, Papulaspora equi, and Scytalidium lignicola), 5 hyaline (colorless) fungi (Aspergillus tamarii, Fusarium chlamydosporum, Fusarium incarnatum, Fusarium lichenicola, and Fusarium sacchari), and one yeast (Trichosporon asahii). Amphotericin B had good in vitro activity (minimum inhibitory concentration [MIC] ≤1 µg/mL) against most dematiaceous fungi, but not hyaline fungi (MIC ≥1 µg/mL). Natamycin showed variable MIC, itraconazole and voriconazole had good in vitro activity, except in Fusarium species. Alternaria tenuissima and A. fusispora had a very high MIC (≥16 µg/mL) against echinocandins. Literature search revealed 27 FK cases due to F. lichenicola (n = 6), P. equi (n = 5), F. sacchari (n = 4), A. fusispora (n = 3), S. lignicola (n = 2), and others (n = 7), and more than 50% of these were reported from India. CONCLUSIONS: Plant fungal pathogens with variable antifungal susceptibility are an emerging cause of human keratitis with predominance of dematiaceous fungi. Identification and antifungal susceptibility testing are important for epidemiology and to optimize therapy and improve the patient outcome.


Assuntos
Infecções Oculares Fúngicas/microbiologia , Fungos/isolamento & purificação , Hialina , Ceratite/microbiologia , Centros de Atenção Terciária/estatística & dados numéricos , Infecções Oculares Fúngicas/epidemiologia , Humanos , Incidência , Índia/epidemiologia , Ceratite/epidemiologia
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