Primary pleomorphic liposarcoma of the orbit: a case report.

To our knowledge, pleomorphic liposarcoma (PL) of the orbit has only been reported in the literature four times. This rarity makes it more difficult to diagnose and to treat in this clinical setting. A 62-year-old female presented with pruritus, edema, proptosis and diplopia 5 months OS. Imaging revealed an intraorbital mass displacing the globe, with infiltration into the sinus. The tumor was removed and the histological examination revealed a highly cellular tumor with heterogenous histology, with a few vacuolated cells and many malignant features. Immunohistochemistry allowed for the differential diagnosis, resulting in a diagnosis of PL of the orbit. The cells were immuno-positive for S-100 and negative for all other relevant markers. According to the literature, prognosis for this neoplasm is quite poor, and exenteration represents the best treatment option. The patient refused exenteration and radiation therapy, however, at 2 year follow-up, she remained recurrence-free.

Oncostatin M receptor-beta mutations underlie familial primary localized cutaneous amyloidosis.

Familial primary localized cutaneous amyloidosis (FPLCA) is an autosomal-dominant disorder associated with chronic skin itching and deposition of epidermal keratin filament-associated amyloid material in the dermis. FPLCA has been mapped to 5p13.1-q11.2, and by candidate gene analysis, we identified missense mutations in the OSMR gene, encoding oncostatin M-specific receptor beta (OSMRbeta), in three families. OSMRbeta is a component of the oncostatin M (OSM) type II receptor and the interleukin (IL)-31 receptor, and cultured FPLCA keratinocytes showed reduced activation of Jak/STAT, MAPK, and PI3K/Akt pathways after OSM or IL-31 cytokine stimulation. The pathogenic amino acid substitutions are located within the extracellular fibronectin type III-like (FNIII) domains, regions critical for receptor dimerization and function. OSM and IL-31 signaling have been implicated in keratinocyte cell proliferation, differentiation, apoptosis, and inflammation, but our OSMR data in individuals with FPLCA represent the first human germline mutations in this cytokine receptor complex and provide new insight into mechanisms of skin itching.

Eyelid and conjunctival paracoccidioidomycosis simulating carcinoma.

Paracoccidioidomycosis (PCM) is the most prevalent systemic mycosis in immunocompetent individuals in Brazil. Ocular infection by PCM is rare; however, when infection does occur, the most common ocular sites involved are eyelid and conjunctiva. A 68-year-old white male presented with a 2-month history of a painless, ulcerated, infiltrative and diffuse whitish lesion located on the right inferior eyelid. A clinical diagnosis of malignant tumor, possibly squamous cell carcinoma, was made. The histopathologic examination showed a hyperplastic epithelium with inflammatory infiltrate of lymphocytes, plasma cells, neutrophils and histiocytes. Large numbers of giant cells were also present. Periodic acid Schiff and Grocott (silver methenamine) stains showed several large round structures with peripheral lateral small budding cells that resembled a "ship's wheel". No multinucleated fungi were seen. The fungi varied in size and small forms were round and single fungal structures. A diagnosis of paracoccidioidomycosis was made PCM eyelid infection is rare and can simulate carcinoma both clinically and histopathologically.

Infiltrating syringomatous adenoma of the nipple.

Infiltrating syringomatous adenoma of the nipple is a rare, benign, locally invasive tumor with recurrence potential, showing sweat duct differentiation. It can clinically, radiologically and pathologically mimic cancer. Histopathologically, it must be distinguished from florid papillomatosis, adenosquamous carcinoma, adenoid cystic carcinoma and sclerosing syringomatous carcinoma. A 44-year-old woman presented with pain on the right nipple for 7 days. On physical exam there was an irregular nodule on nipple area with edema. The skin was intact. The ultrasound showed a hypoechoic irregular nodule measuring 7.5 mm in the nipple area. The mammography was unspecific. The lesion was surgically removed and histopathologically, the tumor was composed of ducts and tubules lined with a double-layered epithelial cells. The lining cells were small, cuboidal with a central nuclei and eosinophilic nuclei. The stroma was dense with lymphocytes and plasma cells, and compressed many of the ducts that contained a comma or tadpole-shape, giving an impression of a syringoma. Some ducts were slightly dilated with squamous metaplasia. Some of these cysts were connected with the overlying epidermis. Mitotic figures were rare and no pleomorfism or hyperchromasia was observed. At the periphery, the ducts invaded muscular fibers of the nipple. The surgical margins were free of neoplastic involvement. Patient has no signs of progression of disease in 1 year of follow-up.

The use of resorcine adhesive in repairing osteochondral's defect in knees of rabbits.

PURPOSE: To study the morphology of the articulation of the knee of rabbits after the repairing of the defect osteochondral standardized with resorcina adhesive or metallic synthesis. METHODS: The procedure was to the creation of the defect osteochondral in femoral medial condylus of the knee of 80 rabbits, The animals were distributed in two groups with continuations of 7 and 42 days and submitted to the technique G (resection and retreat of the fragment osteochondral of the femoral medial condylus and relocation with resorcina adhesive), technique S (resection and retreat of the fragment osteochondral of the femoral medial condylus and relocation and metallic synthesis) or technique C (resection and retreat of the fragment osteochondral of the femoral medial condylus, leaving the empty standard defect the control). It was Made clinical study, radiographic, macroscopic and histological in two groups. RESULTS: the resorcina adhesive provokes: necrosis of the fragment osteochondral in 100% and 95%, degeneration 90% and 100%, free body in 80% and 65% respectively in the group I and II; compared with the metallic synthesis that it presented: necrosis in 25% and 35%, degeneration 25% and 35%, free body in 35% and 10% respectively in the group I and II. CONCLUSION: the resorcinol adhesive, related with the necrosis, cartilaginous degeneration and detachment of the fragment osteochondral lives frequently that the metallic synthesis.

Treatment of fistula-in-ano with cyanoacrylate glue with and without prior seton placement in rats.

PURPOSE: To evaluate the efficacy of ethyl-cyanoacrylate in the treatment of fístula-in-ano in rats with and without prior seton placement. METHODS: Thirty rats Wistar with fístula-in-ano produced surgically, distributed in three groups: group A (ethyl-cyanoacrylate) - treated by application of ethyl-cyanoacrylate into fístula tract; group B (seton + ethyl-cyanoacrylate) - seton placement followed by application of ethyl-cyanoacrylate into fístula tract, 30 days later; group C (control) - no treatment. After 60 days the animals were submited to euthanasia and the specimens were analyzed by pathologist. The results were analyzed by Chi-square test with significant value of p<0.05. RESULTS: One animal of group B died. Were found tracts fully healed: 7, 5 and 2, in groups A, B and C, respectivelly. There was a statistically significant difference between groups A and C (p=0.02). When joined all animals treated with glue (A + B) compared to group C there was also statistically significant difference (p=0.02). CONCLUSIONS: The use of ethyl-cyanoacrylate glue was effective in closing anal fistulae in rats. There was no advantage in the prior application of seton.

Melanoma inhibitory activity in Brazilian patients with cutaneous melanoma.

BACKGROUND: Melanoma inhibitory activity is a protein secreted by melanoma cells and has been used as a tumor marker. Increased Melanoma inhibitory activity serum levels are related to metastatic disease or tumor recurrence. Currently there are no studies on Melanoma inhibitory activity and cutaneous melanoma involving Brazilian patients. OBJECTIVE: To evaluate the performance and feasibility of measuring Melanoma inhibitory activity levels in Brazilian patients with cutaneous melanoma. METHODS: Blood was obtained from ten patients with proved metastatic cutaneous melanoma (Group 1), 15 patients resected for cutaneous melanoma without metastasis (Group 2) and 5 healthy donors (Group 3). Melanoma inhibitory activity was measured using a commercially available ELISA kit. RESULTS: There was a statistically significant difference of Melanoma inhibitory activity levels between patients with and without metastasis (p=0.002), and between patients with metastasis and healthy donors (p=0.002). There was no difference between patients without metastasis and healthy donors (p=0.443). CONCLUSION: Melanoma inhibitory activity is a tumor marker for cutaneous melanoma and the Melanoma inhibitory activity-ELISA test can be easily performed. Patients with metastasis have increased Melanoma inhibitory activity serum levels when compared to patients without metastasis and healthy donors.

Hypoxia-inducible factor-1α and its role in the proliferation of retinoblastoma cells.

In order to better understand the role of HIF-1α in the proliferation of the retinoblastoma cells, a siRNA knockdown of HIF-1α followed by a proliferation assay was performed. Further sequencing was then carried out in order to assess knockdown efficiency and expression of HIF-1α. Upregulation of HIF-1α gene expression in CoCl2-treated retinoblastoma cells was demonstrated via melting curve analysis from PCR tests and was further analyzed using western blot and densitometry analysis. Reduction of HIF-1α expression in retinoblastoma, post HIF-1α knockdown, was observed after siRNA transfection into Y-79 cells. Knockdown of HIF-1α resulted in a significant decrease in proliferation thereby demonstrating that HIF-1α is involved in promoting survival and proliferation in retinoblastoma cells. Stabilization of HIF-1α in retinoblastoma cells using CoCl2 was unsuccessful.

Current and emerging treatment options for uveal melanoma.

Uveal melanoma (UM) is the most common primary malignant intraocular tumor in adults, with a 10-year cumulative metastatic rate of 34%. The most common site of metastasis is the liver (95%). Unfortunately, the current treatment of metastatic UM is limited by the lack of effective systemic therapy. Options for the management of the primary intraocular tumor include radical surgery as well as conservative treatments in order to preserve visual acuity. For metastatic disease, several approaches have been described with no standard method. Nevertheless, median survival after liver metastasis is poor, being around 4-6 months, with a 1-year survival of 10%-15%. In this review, the authors summarize current and promising new treatments for UM.

Unifocal bone paracoccidioidomycosis, Brazil.

We report a 47 year-old man from the State of Mato Grosso do Sul, Brazil, with unifocal tibial paracoccidioidomycosis. A radiograph showed an osteolytic lesion on the tibial middle third diaphysis. The diagnosis was confirmed by histopathologic analysis, and treatment with sulfamethoxazole and trimethoprim was started. After three months, the patient showed significant clinical improvement. Ten months after treatment, the patient showed lesion bone healing. This case describes the rare occurrence of an osteolytic lesion caused by endemic Paracoccidioides brasiliensis in Latin America.

Treatment of fistula-in-ano with cyanoacrylate glue with and without prior seton placement in rats

ABSTRACT PURPOSE: To evaluate the efficacy of ethyl-cyanoacrylate in the treatment of fístula-in-ano in rats with and without prior seton placement. METHODS: Thirty rats Wistar with fístula-in-ano produced surgically, distributed in three groups: group A (ethyl-cyanoacrylate) - treated by application of ethyl-cyanoacrylate into fístula tract; group B (seton + ethyl-cyanoacrylate) - seton placement followed by application of ethyl-cyanoacrylate into fístula tract, 30 days later; group C (control) - no treatment. After 60 days the animals were submited to euthanasia and the specimens were analyzed by pathologist. The results were analyzed by Chi-square test with significant value of p<0.05. RESULTS: One animal of group B died. Were found tracts fully healed: 7, 5 and 2, in groups A, B and C, respectivelly. There was a statistically significant difference between groups A and C (p=0.02). When joined all animals treated with glue (A + B) compared to group C there was also statistically significant difference (p=0.02). CONCLUSIONS: The use of ethyl-cyanoacrylate glue was effective in closing anal fistulae in rats. There was no advantage in the prior application of seton.

Melanocytoma of ciliary body and choroids simulating melanoma.

Melanocytoma is a rare intraocular tumor. There are some reports in the literature dealing with primary melanocytomas of the choroid and ciliary body. It is believed that most of these tumors are clinically diagnosed as nevi or melanoma, and are followed up or treated without surgical resection, respectively. Some clinical features can give a clue as to the correct diagnosis. We report on a 47-year-old white female with progressive visual loss of 2 months and right painful eye. Her visual acuity of finger counting was confined to 3.0m OD and 20/20 OS. Biomicroscopy OD showed a 360 degrees posterior synechia, and fundoscopy was not conclusive due to vitreous opacity. No alterations were seen on OS. Intraocular pressure was normal, and the pupillary reflex was present in both eyes. An ultrasound of the OD showed an elevated tumor on topography of the ciliary body and anterior choroid at the ora serrata level. Melanoma was the main diagnosis considered, and enucleation was indicated due to poor prognosis for visual acuity. Gross and histopathologic examinations of the OD showed a heavily pigmented tumor. The brownish pigment obscured the morphology of the tumor cells that could not be visualized by conventional H&E stain. Bleached slides showed that tumor was composed of melanocytoma cells type I.

Melanoma inhibitory activity in Brazilian patients with cutaneous melanoma

Abstract BACKGROUND: Melanoma inhibitory activity is a protein secreted by melanoma cells and has been used as a tumor marker. Increased Melanoma inhibitory activity serum levels are related to metastatic disease or tumor recurrence. Currently there are no studies on Melanoma inhibitory activity and cutaneous melanoma involving Brazilian patients. OBJECTIVE: To evaluate the performance and feasibility of measuring Melanoma inhibitory activity levels in Brazilian patients with cutaneous melanoma. METHODS: Blood was obtained from ten patients with proved metastatic cutaneous melanoma (Group 1), 15 patients resected for cutaneous melanoma without metastasis (Group 2) and 5 healthy donors (Group 3). Melanoma inhibitory activity was measured using a commercially available ELISA kit. RESULTS: There was a statistically significant difference of Melanoma inhibitory activity levels between patients with and without metastasis (p=0.002), and between patients with metastasis and healthy donors (p=0.002). There was no difference between patients without metastasis and healthy donors (p=0.443). CONCLUSION: Melanoma inhibitory activity is a tumor marker for cutaneous melanoma and the Melanoma inhibitory activity-ELISA test can be easily performed. Patients with metastasis have increased Melanoma inhibitory activity serum levels when compared to patients without metastasis and healthy donors. .

Anatomopathological study of cardiomyopathy induced by doxorubicin in rats.

PURPOSE: The development of an experimental model of myocardiopathy induced by Doxorubicin in rats. METHODS: 16 wistar male rats were randomized in two groups: Group I (placebo) and Group II (Doxorubicin - 5mg/kg). After six months, the animals were subjected to cardiotomy and their hearts were weighted and submitted to transversal cuts, from which fragments for a macro and micro study were obtained. These fragments were studied considering their external and internal diameters and the thickness of the left ventricle (LV). The histological pieces were analyzed for the presence of fibrosis, cytoplasmic vacuolization, necrosis and size of nucleus variation. Data obtained was submitted to statistical analysis with Student's t test. RESULTS: The hearts of the animals in Group II increased 41% in relation to their weight; 33% in the internal diameter and 14% in the external diameter of the LV cavity; and 24% in the thickness of the wall. Fibrosis of the myocardial tissue was observed in 75% of the animals of Group II; all the animals presented miocyte cytoplasmatic vacuolization; myocardial necrosis was present in 75% of the animals; and 87/% presented variation in the size of myocite nuclei. The presence of polymorphonuclear cells was also observed. CONCLUSION: Doxorubicin was effective in the promotion of macro and microscopic alterations in the cardiac tissue of rats, possibly constituting a model for the experimental study of myocardiopathy.

Familial primary localized cutaneous amyloidosis in Brazil.

BACKGROUND: Macular and lichen amyloidosis are clinical variants of primary localized cutaneous amyloidosis (PLCA). Most cases are sporadic, but approximately 10% of cases may be familial. To our knowledge, the clinicopathologic and molecular features of such pedigrees, however, have not been studied in detail. OBSERVATIONS: We assessed 2 Brazilian families with either lichen-type (family 1 had 14 affected subjects) or macular-type (family 2 had 7 affected subjects) PLCA. Typically, in both pedigrees, the onset of symptoms was around puberty, and pruritus usually began on the lower legs. Findings from lesional skin biopsy samples from both families showed thioflavin T-positive material in the papillary dermis, which was more prominent in the lichen phenotype in family 1. Spontaneous improvement occurred in 3 subjects (from both families) after age 25 years. All affected individuals in family 1 had a heterozygous missense mutation in the OSMR gene (p.I691T), but no pathogenic mutation in OSMR was found in family 2. CONCLUSIONS: Familial PLCA shows autosomal dominant inheritance, but there is clinical and genetic heterogeneity and variable clinical penetrance. Demonstration of mutations in the OSMR gene provides new insight into mechanisms of itch and apoptosis in human skin.

Anatomopathological study of cardiomyopathy induced by doxorubicin in rats / Estudo anatomopatológico da miocardiopatia induzida pela doxorrubicina em ratos

Purpose: The development of an experimental model of myocardiopathy induced by Doxorubicin in rats. Methods: 16 wistar male rats were randomized in two groups: Group I (placebo) and Group II (Doxorubicin - 5mg/kg). After six months, the animals were subjected to cardiotomy and their hearts were weighted and submitted to transversal cuts, from which fragments for a macro and micro study were obtained. These fragments were studied considering their external and internal diameters and the thickness of the left ventricle (LV). The histological pieces were analyzed for the presence of fibrosis, cytoplasmic vacuolization, necrosis and size of nucleus variation. Data obtained was submitted to statistical analysis with Student's t test. Results: The hearts of the animals in Group II increased 41 percent in relation to their weight; 33 percent in the internal diameter and 14 percent in the external diameter of the LV cavity; and 24 percent in the thickness of the wall. Fibrosis of the myocardial tissue was observed in 75 percent of the animals of Group II; all the animals presented miocyte cytoplasmatic vacuolization; myocardial necrosis was present in 75 percent of the animals; and 87/ percent presented variation in the size of myocite nuclei. The presence of polymorphonuclear cells was also observed. Conclusion: Doxorubicin was effective in the promotion of macro and microscopic alterations in the cardiac tissue of rats, possibly constituting a model for the experimental study of myocardiopathy.

Objetivo: Desenvolver um modelo experimental de miocardiopatia induzida por doxorrubicina em ratos. Métodos: 16 ratos Wistar machos foram randomizados em 2 grupos: Grupo I (placebo) e Grupo II (doxorrubicina 5mg/kg). Após 6 meses, os animais foram submetidos a cardiotomia e seus corações foram pesados e submetidos a cortes transversais. Estes fragmentos foram estudados considerando seus diâmetros externos e internos e a espessura do ventrículo esquerdo. As peças histológicas foram analisadas quanto à presença de fibrose, vacuolização citoplasmática, necrose e variação do tamanho do núcleo. Os resultados foram submetidos a análise estatística pelo teste t de Student. Resultados: Os corações dos animais do grupo II aumentaram 41 por cento em relação ao peso; 33 por cento no diâmetro interno e 14 por cento no diâmetro externo; e 24 por cento na espessura da parede do VE. Fibrose do tecido miocárdico foi observada em 75 por cento dos animais do grupo II; todos os animais apresentaram vacuolização citoplasmática dos miócitos; Houve necrose miocárdica em 75 por cento dos animais e 87 por cento apresentaram variação no tamanho do núcleo. A presença de células polimorfonucleares também foi observada. Conclusão: A doxorrubicina foi efetiva na promoção de alterações macro e microscópicas no tecido cardíaco de ratos, possivelmente constituindo-se num modelo experimental para estudo da miocardiopatia.

The use of resorcine adhesive in repairing osteochondral's defect in knees of rabbits

PURPOSE: To study the morphology of the articulation of the knee of rabbits after the repairing of the defect osteochondral standardized with resorcina adhesive or metallic synthesis. METHODS: The procedure was to the creation of the defect osteochondral in femoral medial condylus of the knee of 80 rabbits, The animals were distributed in two groups with continuations of 7 and 42 days and submitted to the technique G (resection and retreat of the fragment osteochondral of the femoral medial condylus and relocation with resorcina adhesive), technique S (resection and retreat of the fragment osteochondral of the femoral medial condylus and relocation and metallic synthesis) or technique C (resection and retreat of the fragment osteochondral of the femoral medial condylus, leaving the empty standard defect the control). It was Made clinical study, radiographic, macroscopic and histological in two groups. RESULTS: the resorcina adhesive provokes: necrosis of the fragment osteochondral in 100 percent and 95 percent, degeneration 90 percent and 100 percent, free body in 80 percent and 65 percent respectively in the group I and II; compared with the metallic synthesis that it presented: necrosis in 25 percent and 35 percent, degeneration 25 percent and 35 percent, free body in 35 percent and 10 percent respectively in the group I and II. CONCLUSION: the resorcinol adhesive, related with the necrosis, cartilaginous degeneration and detachment of the fragment osteochondral lives frequently that the metallic synthesis.

OBJETIVO: Estudar a morfologia da articulação do joelho de coelhos após a reparação de um defeito osteocondral padronizado com adesivo de resorcina ou síntese metálica. MÉTODOS: Procedeu-se à criação de um defeito osteocondral em côndilo femoral medial do joelho de 80 coelhos. Os animais foram distribuídos em dois grupos com seguimentos de 7 e 42 dias e submetidos à técnica G (ressecção e retirada do fragmento osteocondral do côndilo femoral medial e recolocação com adesivo de resorcina), técnica S (ressecção e retirada do fragmento osteocondral do côndilo femoral medial e recolocação e síntese metálica) ou técnica C (ressecção e retirada do fragmento osteocondral do côndilo femoral medial, deixando o defeito padrão vazio como controle). Fez-se estudo clínico, radiográfico, macroscópico e histológico nos dois grupos. RESULTADOS: o adesivo de resorcina provoca: necrose do fragmento osteocondral em 100 por cento e 95 por cento, degeneração 90 por cento e 100 por cento, corpo livre em 80 por cento e 65 por cento respectivamente no grupo I e II; comparado com a síntese metálica que apresentou: necrose em 25 por cento e 35 por cento, degeneração 25 por cento e 35 por cento, corpo livre em 35 por cento e 10 por cento respectivamente no grupo I e II. CONCLUSÃO: o adesivo de resorcina, está relacionado com a necrose, degeneração cartilaginosa e despreendimento do fragmento osteocondral com maior freqüência que a síntese metálica.

Angiomixoma agressivo em paciente do sexo masculino acometendo cordão espermático: relato de caso e revisão da literatura / Aggressive angiomyxoma in male patient on the spermatic cord: case report and literature review

Os autores relatam um caso de angiomixoma agressivo em paciente do sexo masculino, no cordão espermático. Essa entidade é descrita predominantemente em mulheres adultas, acometendo as regiões perineal, genital e pélvica. Macroscopicamente observou-se massa lobulada, infiltrativa, de limites imprecisos, que microscopicamente era representada por estroma mixóide com células pequenas, fusiformes e/ou estreladas, uniformes, sem figuras de mitose evidentes. Chamou atenção a presença de vasos sangüíneos proeminentes, de variados tamanhos, alguns deles com paredes espessadas. O perfil imuno-histoquímico mostrou positividade focal para desmina e actina de músculo liso (1A4) e negatividade para CD34 e CD68. Muitas neoplasias mixóides, tanto benignas quanto malignas, podem ser confundidas com o angiomixoma agressivo. O diagnóstico diferencial deve ser amplamente estudado, pois essa neoplasia tem caráter infiltrativo, alto índice de recorrência, embora não haja metástases relatadas até o presente momento.