Vacuoles in muscle fiber: pathological differences in various diseases / 中华神经科杂志

The vacuole in muscle fibers is a non-specific myopathological change. As a myopathological term, it occurs in several rare disorders. The vacuoles are mostly related to the autophagy of glycogen, lipids, abnormal proteins, and organelles, and a few are caused by glycogen or lipids deposition or sarcoplasmic reticulum luminal vacuolization. The vacuoles impair the structure of muscle fibers, being one of the myopathological features in various diseases. In this review, clinical features and myopathological changes of various diseases with vacuolar muscle fibers were introduced and the diagnostic value of intrafibral vacuoles was focused on. Knowing the underlying pathogenesis is required to understand these myopathological changes.

Evidence of autophagic vesicles in a patient with Lisch corneal dystrophy / Evidência de vesículas autofágicas em paciente com distrofia corneana de Lisch

ABSTRACT Lisch corneal dystrophy is a rare corneal disease characterized by the distinctive feature of highly vacuolated cells. Although this feature is important, the nature of these vacuoles within corneal cells remains unknown. Here, we sought to analyze corneal cells from a patient diagnosed with Lisch dystrophy to characterize the vacuoles within these cells. Analyses using histopathology examination, confocal microscopy, and transmission electron microscopy were all consistent with previous descriptions of Lisch cells. Importantly, the vacuoles within these cells appeared to be autophagosomes and autolysosomes, and could be stained with an anti-microtubule-associated protein 1A/1B-light chain 3 (LC3) antibody. Taken together, these findings indicate that the vacuoles we observed within superficial corneal cells of a patient with Lisch corneal dystrophy constituted autophagosomes and autolysosomes; this finding has not been previously reported and suggests a need for further analyses to define the role of autophagy in this ocular disease.

RESUMO A distrofia corneana de Lisch é uma doença rara, caracterizada principalmente pela presença de células altamente vacuoladas. Embora esta característica seja importante, a natureza desses vacúolos dentro das células da córnea permanece des conhecida. Aqui, procuramos analisar as células da córnea de um paciente diagnosticado com distrofia de Lisch para caracte rizar os vacúolos dentro dessas células. Análises utilizando exame histopatológico, microscopia confocal e microscopia eletrônica de transmissão foram todas consistentes com descrições previas de células de Lisch. Importante, os vacúolos dentro dessas células pareciam ser autofagossomos e autolisossomos, e po deriam ser corados com um anticorpo proteico 1A/1B-cadeia leve 3 (LC3) da proteína anti-microtúbulo associado a microtúbulos. Em conjunto, esses achados indicam que os vacúolos observados nas células superficiais da córnea de um paciente com distrofia corneana de Lisch constituíram autofagossomos e autolisossomos. Esse achado não foi relatado anteriormente e sugere a necessidade de mais análises para definir o papel da autofagia nessa doença ocular.

Ultrastructure of intracytoplasmic Rickettsia-like infection of the gills of the teleost Archosargus probatocephalus (Sparidae) in northeastern Brazil / Ultraestrutura de Rickettsia intracitoplasmática infectando as brânquias do teleósteo Archosargus probatocephalus (Sparidae) no Nordeste do Brasil

Abstract A histopathological survey was conducted to investigate the presence of microparasites in fish Archosargus probatocephalus in a river near Maceió, Brazil. Light microscope observations of fragments of gill showed the presence of small cysts containing numerous myxospores that were morphologically identified as Henneguya. Transmission electron microscopy observations further revealed several gill cells containing groups of prokaryotic cells within large cytoplasmic vacuoles. Each infected host cell displayed a single vacuole containing a variable number of Rickettsia-like cells (up to 11), some of which presented the dumbbell shape characteristic of binary fission. The Rickettsia-like cells were pleomorphic, without a nucleus and with chromatin dispersed in the cytoplasm. They had a thin electron-dense wall of Gram-negative type. The morphology of these prokaryotic was similar to those of the order Rickettsiales and was described as a Rickettsia-like organism. Histopathological evaluation showed that several vacuole membranes had a lysed appearance. Some had ruptured, thus allowing direct contact between the Rickettsia-like organism and the cytoplasm of the host cell. The rupturing of the branchial epithelium may have contributed towards reduction of the surface area of the gills, but it is not possible to say that this was the cause of the host's death.

Resumo Um levantamento histopatológico foi realizado para pesquisar a presença de microparasitas, no peixe Archosargus probatocephalus, em um rio próximo a Maceió, Brasil. Observações ao microscópio óptico de fragmentos de brânquias mostraram a presença de pequenos cistos contendo numerosos mixósporos, identificados morfologicamente como Henneguya. Ocasionalmente, na microscopia eletrônica de transmissão, foram observados vários corpos citoplasmáticos de inclusão, grupo aparentemente de células procarióticas que vivem dentro de um grande vacúolo citoplasmático de algumas células branquiais. As células hospedeiras infectadas tinham um único vacúolo contendo um número variável de células do tipo Rickettsia, até 11, algumas das quais em forma do haltere, característica da fissão binária. Essas células eram pleomórficas sem núcleo, tendo a cromatina dispersa no citoplasma e possuíam uma parede densa de elétrons finos do tipo Gram-negativo. A morfologia dessas células procarióticas foi semelhante àquelas da ordem Rickettsiales e foram descritas como organismos tipo Rickettsiae. A histopatologia mostra várias membranas de vacúolos circundantes com aspetos lisados, enquanto outras apresentam rupturas que mostram contato direto do organismos tipo Rickettsiae com o citoplasma da célula hospedeira. A ruptura do epitélio branquial pode ter contribuído para a redução da superfície das brânquias, mas não é possível afirmar que foi a causa da morte do hospedeiro.

Clinical, muscle imaging and electrophysiological features of oculopharyngodistal myopathy / 中国神经精神疾病杂志

Objective To investigate the clinical manifestation and electrophysiological, muscle imaging and pathological, molecular features of oculopharyngodistal myopathy (OPDM). Methods The clinical electrophysiological, muscle imaging and pathological, molecular data was collected from a case of OPDM. Data analysis was conducted together with a literature. Results The onset age of the patient was 25 years old. The sequential order of involved muscle was upper eyelid muscle, external ocular, laryngopharyngeal, facial, distal limb muscle and proximal upper limb. Serum creatine kinase was mildly elevated. Electromyography revealed myogenic changes with demyelinating peripheral neuropathy. Myopathological findings showed myopathic changes with rimmed vacuoles . Muscle image showed that fatty replacement of was more severe in lower legs than in thigh. Posterior muscle was severely involved in lower legs. All known genes responsible for distal and myofibrillar myopathies, vacuolar myopathies, and muscular dystrophies were excluded by targeted next-generation sequencing. Conclusion The case is a sporadic case. OPDM is a disease with a unique phenotype which not only affects muscle but also involves multiple system (demyelinating peripheral neuropathy、heart disease and so on).

Novel Mutation of the GNE Gene Presenting Atypical Mild Clinical Feature: A Korean Case Report

Glucosamine (UDP-N-acetyl)-2-epimerase/N-acetylmannosamine kinase (GNE) myopathy is caused by mutations in GNE, a key enzyme in sialic acid biosynthesis. Here, we reported a case of GNE that presented with atypical mild clinical feature and slow progression. A 48-year-old female had a complaint of left foot drop since the age of 46 years. Electromyography (EMG) and muscle biopsy from left tibialis anterior muscle were compatible with myopathy. Genetic analysis led to the identification of c.1714G>C/c.527A>T compound heterozygous mutation, which is the second most frequent mutation in Japan as far as we know. Previous research has revealed that c.1714G>C/c.527A>T compound heterozygous mutation is a mild mutation as the onset of the disease is much later than the usual age of onset of GNE myopathy and the clinical course is slowly progressive. This was the first case report in Korea of the clinicopathological characteristics of GNE myopathy with GNE (c.1714G>C/c.527A>T compound heterozygous) mutation.

The role of suppression of p38 MAPK in cellular vacuole formation / 重庆医学

Objective To investigate the role of the p38 MAPK pathway in the formation of cytoplasmic vacuoles .Methods Af-ter treated with Anisomycin ,SB203580 or SP600125 ,images of HepG2 ,LM3 ,QBC939 ,Hela and A549 cells were recorded by light microscopy and taken at a magnification of 400 × .The effects of anisomycin ,SB203580 and SP600125 on the activity of p38 and JNK were measured by Western blot .LM3 and A549 cells were stained with the ER-tracker red and the lyso-tracker red and subjec-ted to confocal microscopy analysis .Results (1)Anisomycin could abolish cytoplasmic vacuolization of HepG2 cells .(2)p38 MAPK activation was responsible for anisomycin-induced cytoplasmic vacuolization abolishment .(3)p38 MAPK blocking initiated cytoplas-mic vacuoles formation in various cancer cell lines .(4)p38 MAPK blocking-induced cytoplasmic vacuoles disrupted the integrity of endoplasmic reticulum .(5)p38 MAPK blocking reversibly induced cytoplasmic vacuoles formation .Conclusion These observations provide direct evidence for a role of p38 MAPK signaling in regulating the formation of cytoplasmic vacuoles .

Muscle biopsy in Pompe disease / Biópsia muscular na doença de Pompe

Pompe disease (PD) can be diagnosed by measuring alpha-glucosidase levels or by identifying mutations in the gene enzyme. Muscle biopsies can aid diagnosis in doubtful cases. Methods: A review of muscle biopsy from 19 cases of PD (infantile, 6 cases; childhood, 4 cases; and juvenile/adult, 9 cases). Results: Vacuoles with or without glycogen storage were found in 18 cases. All cases had increased acid phosphatase activity. The vacuole frequency varied (almost all fibers in the infantile form to only a few in the juvenile/adult form). Atrophy of type 1 and 2 fibers was frequent in all forms. Atrophic angular fibers in the NADH-tetrazolium reductase and nonspecific esterase activity were observed in 4/9 of the juvenile/adult cases. Conclusion: Increased acid phosphatase activity and vacuoles were the primary findings. Most vacuoles were filled with glycogen, and the adult form of the disease had fewer fibers with vacuoles than the infantile or childhood forms. .

O diagnóstico da doença de Pompe (PD) pode ser feito pela dosagem da enzima alfa-glicosidase ou pela mutação do seu gene codificador. A biópsia muscular pode ajudar em casos duvidosos. Métodos: Revisão das biópsias musculares de 19 casos de PD (forma infantil, 6 casos; infantil tardia, 4; e juvenil/adulto, 9). Resultados: Encontrados vacúolos em 18 casos, com ou sem depósito de glicogênio. Todos mostraram aumento da fosfatase ácida. Os vacúolos estavam presentes na maioria das fibras nas formas infantis, menos frequentes nas formas juvenil e mais raros nas formas do adulto. A atrofia de fibras dos tipos 1 e 2 ocorreram em todas as formas. Fibras atróficas na NADH-tetrazolium redutase e esterase não específica foram observadas em 4/9 das formas infantil tardia/adulta. Conclusões: Os dados mais frequentes foram vacúolos, preenchidos por glicogênio com atividade aumentada da fosfatase ácida. A forma adulta apresenta menor número de vacúolos que as formas infantil e infantil tardia. .

Approach to the patient with empty sella combined with adrenal adenoma / 中华内分泌代谢杂志

Clinical characteristics were analyzed retrospectively in a patient with empty sella and adrenal adenoma with regard to the elaborated diagnosis and treatment.Although empty sella syndrome alone was common,clinical combination with adrenal adenoma was rarely reported.It was difficult to diagnose due to complex symptoms and hormone levels.This case could help increase awareness of the disease and accumulate the experience in diagnosis and treatment.

Histogenesis of Developing Human Thyroid.

In human beings the thyroid gland is one of the largest of the endocrine organs. It is one of the earliest endocrine organs to be differentiated and has an important hormonal role in embryonic development. The importance of thyroid gland is to promote growth and development of the brain during fetal life and for the first few years of post-natal life[1, 2]. The purposes of present study are 1)to study the microscopic structure of the human thyroid in different gestational age groups of normal stillborn foetuses; 2) to correlate the size of thyroid follicles; the nature and amount of colloid content of thyroid follicles at different stages of development. The study was carried out on 50 stillborn normal human foetuses. The microscopic structure of thyroid was studied under light microscope. The study concluded the developmental staging of thyroid as: the precolloid stage; the colloid formation stage; the folliculogenesis stage; secretory activity stage.

Dysferlinopathy: Spectrum of pathological changes in skeletal muscle tissue.

Background: Dysferlinopathy is an autosomal recessive-limb girdle muscular dystrophy (AR-LGMD) caused due to the defect in gene encoding dysferlin, a sarcolemmal protein. Awareness of the variants and their relative frequency is essential for accurate diagnosis. Aim: To study the spectrum of morphologic changes in immunohistochemically proven cases of dysferlinopathies, to correlate the findings with clinical phenotype and durations of illness and determine the frequency. Materials and Methods: Dysferlinopathies seen over a period of 2 years at a tertiary neurological center were analyzed. Results: Clinically, majority had Miyoshi phenotype (46.6%) with distal involvement and LGMD phenotype (40%) with proximal muscle involvement. In addition, a proximo-distal and tibial muscle phenotype was encountered. Morphologically, rimmed vacuoles were noted in the Miyoshi phenotype. The presence of ragged red fibers, lobulated fibers and inflammation had no preference to a particular phenotype. Significant atrophy and lobulated fibers were noted in patients with longer duration of illness. Conclusions: Dysferlinopathy was the second most common identifiable cause (21%) of LGMD next to sarcoglycanopathies (27%).

Clinical and pathological features of 20 patients of glycogen storage disease type Ⅱ / 中华神经科杂志

Objective To summarize the clinical and pathological features of glycogen storage disease (GSD) type Ⅱ. Methods The clinical and pathological data of the 20 GSD type Ⅱ patients were reviewed. Results One patient with infantile-onset mainly presented hypotonia, muscle weakness, feeding difficulties, pulmonary infection and cardiomyopathy insufficiency and increase of serum creatine kinase (778 IU/L) and echographic evidence of hypertrophic cardiomyopathy were detected. Electromyography studies indicated a definite myopathy. Nineteen cases were late-onset, presenting a slowly progressive proximal myopathy with truncal involvement or with symptoms dominated by respiratory insufficiency. Not all muscles were equally affected. Increase of serum creatine kinase (208-2600 IU/L) was detected in 14 patients and normal level in 1 patient. Electromyography studies indicated a definite myopathy in 9 patients,with abnormal irritability in 1 patient and susceptible in 4 patients and myotonic discharge in 1 patient and no abnormalities in 2 patients. Echographic evidence of thickening of the interventricular septum and pulmonary hypertension were detected in 2 patients respectively. The common light microscopic feature of all case was a vacuolar myopathy with high glycogen content and acid phosphatase activity in the vacuoles. Conclusions GSD type Ⅱ often presents slowly progressive myopathy which often affect the toro and respiratory muscles.In most patients the serum creatine kinase level is elevated slightly. Muscle biopsy is of use to make the definite diagnosis of this disease.

Dorfman-Chanarin syndrome: A rare neutral lipid storage disease.

Dorfman-Chanarin syndrome is a rare neutral lipid storage disorder characterized by ichthyosis, lipid vacuolations in peripheral leucocytes, and multisystem involvement. It is an autosomal recessive disorder caused by mutations in the CGI-58 gene. A total of 42 cases have been reported worldwide till February 2009 out of which 4 have been previously reported from India. We report a case of a 20-month-old male with congenital ichthyosis, organomegaly, and bilateral cryptorchidism. Examination of the peripheral smear revealed lipid vacuoles in the leucocytes consistent with Jordan's anomaly, which was confirmed by transmission electron microscopy. Liver biopsy revealed micronodular cirrhosis with macrovesicular steatosis while skin biopsy showed ichthyosis vulgaris. Dorfman-Chanarin syndrome was diagnosed on the basis of clinical and laboratory criteria with certain unreported manifestations. Dietary modifications were instituted and followed up after 1 year with promising results. This emphasizes the importance of neonatal screening for lipid vacuolations in peripheral blood in all cases of congenital ichthyosis.

Oculopharyngeal muscular dystrophy,the clinical,electrophysiologic,myopathological and genetic study in a family / 中华神经科杂志

Objective To report clinical,myopathological and genetic features in a family with oculopharyngeal muscular dystrophy(OPMD).Methods The proband,a 60 year-old man,presented proximal weakness of both lower limbs since 50 years old.He developed dysphagia and dysarthria after 53 years old and mild exophthalmos with ptosis after 57 years old.The serum creatine kinase was mildly elevated.Electromyography showed neurogenic involvement and the nerve conduction velocity decreased 20%-143%.Other 5 members in 3 generations developed also dysathria after 45 years old.followed by ptosis 4-20 years afterwards.Three of them showed mild limb weakness.Muscle was biopsied in the proband and specimen was examined with histological,enzymhistochemical,immunohistochemical stainings (first antibody were anti.desmin and ubiquitin antibedies) and ultrastructural examination.PABPN1 gene was sequenced in the proband and 18 family members.Results Rimmed vacuoles with ubiquitin positive material appeared in the muscle fibers.Additionally.there were a few angular atrophic fibers in small groups,COX negative fibers and desmin positive regenerative fibers.Intranuclear palisading filamentous inclusions were observed electromicroscopically in 3% of the nuclears.(GCG)6in PABPN1 was expanded to (GCG)9 in the proband and 11 members.Conclusions The onset symptoms is pharyngeal weakness in OPMD due to heterozygous expanding of PABPNl(GCG)9,accompanied with demyelinating neuropathy.Intranuclear inclusions are also identified in Chinese patient.

Early phase of amyloid beta42-induced cytotoxicity in neuronal cells is associated with vacuole formation and enhancement of exocytosis

Amyloid beta (Abeta) neurotoxicity is believed to play a critical role in the pathogenesis of Alzheimer's disease (AD) mainly because of its deposition in AD brain and its neuronal toxicity. However, there have been discrepancies in Abeta-induced cytotoxicity studies, depending on the assay methods. Comparative analysis of Abeta42-induced in vitro cytotoxicity might be useful to elucidate the etiological role of Abeta in the pathogenesis of AD. In this study, MTT, CCK-8, calcein-AM/EthD-1 assays as well as thorough microscopic examinations were comparatively performed after Abeta42 treatment in a neuronal precursor cells (NT2) and a somatic cells (EcR293). Extensive formation of vacuoles was observed at the very early stage of Abeta42 treatment in both cells. Early observation of Abeta42 toxicity as seen in vacuole formation was also shown in MTT assay, but not in CCK-8 and calcein-AM/EthD-1 assays. In addition, Abeta42 treatment dramatically accelerated MTT formazan exocytosis, implying its effect on the extensive formation of cytoplasmic vacuoles. Abeta42 seems to cause indirect inhibition on the intracellular MTT reduction as well as vacuole formation and exocytosis enhancement. Following the acute cellular dysfunction induced by Abeta42, the prolonged treatment of micromolar concentration of Abeta42 resulted in slight inhibition on redox and esterase activity. The early Abeta42-induced vacuolated morphology and later chronic cytotoxic effect in neuronal cell might be linked to the chronic neurodegeneration caused by the accumulation of Abeta42 in AD patients' brain.

Nonaka Myopathy: A case report

Nonaka myopathy (NM) or distal myopathy with rimmed vacuoles was an autosomal recessive muscle disease with preferential involvement of the tibialis anterior and sparing quadriceps muscles in young adulthood. Patients with NM usually showed slightly elevated serum creatine kinase (CK) levels and characteristic rimmed vacuoles in muscle biopsy. Recently, the UDP-N-acetylglucosamine-2-epimerase/N-ace-tylmannosamine kinase (GNE) gene was identified as the identified as the causative gene for NM. Here we reported a NM patient carrying homozygous mutations (V572L) of the GNE gene. To the best of our knowledge, this was the first report of genetically confirmed NM in Korea and NM should be included in the differential diagnosis of slowly progressive weakness of distal legs.

Distal myopathy with rimmed vacuoles: clinical and pathological study in 9 Chinese cases / 中华神经科杂志

Objective To investigate the clinical and pathological features of distal myopathy with rimmed vacuoles(DMRV). Methods Clinical manifestations and pathological features of biopsied muscle specimens were summarized and analysed retrospectively. Muscle specimens were collected from biceps brachii (3 cases), tibialis anterior (2 cases), quadriceps femoris (1 case)and gastrocnemius (3 cases) respectively. 6 cases were observed by electronic microscope. Results The onset of disease was ranged in age from 15 to 33 years, averaging 25 years. It is more common in women than in men in a ratio of 2∶1. Distal muscle weakness and atrophy of the lower extremities was predominant in early stage. Varied involvement of proximal and trunk muscles, with sparing of the facial, extraocular, bulbar, intercostal and diaphragm muscles was recognized in the advanced stage. The striking and common pathologic finding was the presence of rimmed vacuoles in muscle fibers with little evidence of necrotic or regenerative processes. Electronic microscopic study showed an accumulation of myeloid structure in 5 cases, cytoplasmic inclusion bodies in 4 cases and intranuclear filamentous inclusions in 2 cases. A perforated nuclear envelope and extrusion of filaments in adjacent cytoplasm were found in an enlarged nucleus filled with tubulofilementous inclusion.Conclusion The clinical and pathological findings of DMRV occurred in China is basically similar to those reported by Japanese. Ultrastructural study suggested that sarcoplasmic filamentous inclusions might originate from perforated nuclei with inclusion body, which predicts that the rimmed vacuoles are likely to be the end products of nuclear disintegration and focal myofibrillar degeneration.

Clinical and pathologic features in 71 cases of distal myopathy / 中华神经科杂志

Objective To study the clinical manifestations and pathological features of distal myopathies, we investigated 71 distal myopathy patients collected in the past 16 years.Methods Clinical manifestations and pathological features of biopsied muscle specimens were summarized and analysed retrospectively. Results Twenty-six of the 71 patients were of Nonaka type, 38 of Miyoshi type, 2 of tibial muscular dystrophy (TMD) type, 4 of Welander type, and 1 of oculophayngodistal myopathy (OPDM) type. Nonaka type is a sporadic or autosomal recessively inherited disorder with preferential involvement of the anterior tibial muscles. In the 26 patients with Nonaka myopathy, the onset age ranged from 8 to 39 years, averaging 24 years, and the disease was rather rapidly progressive. Sternocleidomastoid, biceps brachii muscle, pectoralis major muscle and quadriceps femoris were also involved as the disease advanced. The serum CK was slightly elevated or normal. Muscle biopsies showed rimmed vacuoles markedly without obvious dystrophic features. 15-20-nm cytoplasmic and nuclear filaments were usually seen on electron microscope. The patients with Miyoshi type were characterized clinically by sporadic or autosomal recessive inheritance, preferential gastrocnemius muscle involvement, and dystrophic muscle pathology. Rimmed vacuoles were occasionally seen. Average age of onset was 23 years (range of 8-41 years). As the disease advanced, patients with Miyoshi myopathy occasionally showed apparent proximal muscle involvement. Serum CK was markedly elevated, ranging from 3-180 times than the normal. Welander type was found in four cases. Onset age was from 30 to 46 years. Weakness always began in the finger and wrist extensors. As the disease progressed, symptoms were spreading to the distal lower extremities slowly. The serum CK level was normal or only slightly elevated. Muscle biopsies showed dystrophic features, with rimmed vacuoles occasionally. The oneset ages of two patients with TMD were from 41 to 42 years. Weakness was confined mainly to the anterior tibial muscle. Muscle biopsies revealed fibre necrosis and regeneration. Rimmed vacuoles were present significantly. OPDM was found in a 38-year-old-onset patient with autosomal dominant inheritance and characterized by the weakness of distal lower extremity and development of extraocular muscles, vocal cord and pharyngeal muscle weakness. Muscle biopsies showed rimmed vacuoles without fibre necrosis.Conclusion Five types of distal myopathies were present in China, and Miyoshi and Nonaka myopathies were more common. The clinical and pathological findings of Chinese distal myopthies should be basically similar to those reported by other countries.

Four Cases of Multiple Vacuoles inside of Acrysof Intraocular Lens

Acrysof intraocular lens (IOL) (Alcon,Dallas,USA), the first commercially available foldable acrylic IOL, which was approved for marketing in the United states on December, 1994 and in the Korea on August, 1997.It is made with an acrylic/methacrylic polymer. Characteristics of the Acrysofistiguished from commonly used foldable silicon IOLs include improved elastic properties (not slippery when wet), slow and controlled unfolding, and a high refractory index resulting in a thinner lens. We experienced vacuolesin Acrysof IOLs which has no visual influence in 4 cases and report this unusual cases with a review of available literature.

A Case of Sebaceous Carcinoma

Sebaceous carcinoma of meibomian gland is not so common as is generally re ported. In general feature of this tumor may masquerade as a chronic blepharoconjunctivitis or a chalazion for many months before the true diagnosis is esta blished. In the case of orbital involvement secondarily the patient may occasion ally develop as solid nodule in the fossa of the lacrimal gland that may simulate a primary gland tumor, The authors experienced one case of sebaceous carcinoma which developed in the orbital area. This was a 23-rear-old female who had a growing large mass in the left orbital area, which caused proptosis and decreased vision. Computed tomography of the left orbit demonstrated a large soft tissue mass lesion, about 3 X 3 X 1.5 cm in size, in superotemporal side of the retroorbital portion. The patient underwent a lateral orbitotomy and excisional biopsy of the tumor. Microscopic examination showed intracytoplasmic lipoid vacuoles and mixed pattern which was an admixture of lobular and comedocarcinoma-like area. About 2 months later the tumor recurred, so subtotal orbital exenteration with preservation of lid, conjunctiva, cornea and sclera was undertaken and radiotherapy was taken. However, this tumor was extended to brain.

A Case of Sebaceous Carcinoma

Sebaceous carcinoma of meibomian gland is not so common as is generally re ported. In general feature of this tumor may masquerade as a chronic blepharoconjunctivitis or a chalazion for many months before the true diagnosis is esta blished. In the case of orbital involvement secondarily the patient may occasion ally develop as solid nodule in the fossa of the lacrimal gland that may simulate a primary gland tumor, The authors experienced one case of sebaceous carcinoma which developed in the orbital area. This was a 23-rear-old female who had a growing large mass in the left orbital area, which caused proptosis and decreased vision. Computed tomography of the left orbit demonstrated a large soft tissue mass lesion, about 3 X 3 X 1.5 cm in size, in superotemporal side of the retroorbital portion. The patient underwent a lateral orbitotomy and excisional biopsy of the tumor. Microscopic examination showed intracytoplasmic lipoid vacuoles and mixed pattern which was an admixture of lobular and comedocarcinoma-like area. About 2 months later the tumor recurred, so subtotal orbital exenteration with preservation of lid, conjunctiva, cornea and sclera was undertaken and radiotherapy was taken. However, this tumor was extended to brain.