bromochloroacetic-acid and Lacrimal-Apparatus-Diseases

We encountered primary ductal adenocarcinoma of the lacrimal gland in a 67-year-old Japanese man. To the best of our knowledge, only three cases of primary ductal adenocarcinoma of the lacrimal gland have been reported in the literature. The patient was admitted because of visual disturbance, and a mass measuring about 3 cm in diameter was revealed in the right orbit. The mass was resected, and primary ductal adenocarcinoma of the lacrimal gland was diagnosed histopathologically. He died from recurrence at the primary site and metastasis to the brain, lungs, liver, common bile duct, and pancreas 2 years and 10 months after surgery although adjunctive orbital radiotherapy was given. Immunohistochemically, the characteristics of cancer cells were similar to those of salivary duct carcinoma, namely positivity for cytokeratin (CK) 7, 10, 17, 18, 19, and 34betaE12, and negativity for CK20. It was not clear whether the ductal adenocarcinoma originated from the ductal or acinar epithelium of the lacrimal gland, because the immunohistochemical features of both epithelia were identical.

Topics: Aged; Carcinoma, Ductal; Eye Neoplasms; Fatal Outcome; Humans; Immunohistochemistry; Keratins; Lacrimal Apparatus Diseases; Male; Neoplasm Recurrence, Local; Radiotherapy, Adjuvant

Primary poorly differentiated lacrimal gland adenocarcinoma in the orbital region is an extremely rare type of neoplasm with only 1 related case in the literature. Its high grade of malignancy makes the timely data reported necessary. Hence, we present an extremely rare disease with biopsy results and recommendations on clinical treatment in an elderly male with Chinese descent.. A 66-year-old Chinese man presented with swelling in the left ocular region and eyeball proptosis. On physical examination, the patient had redness, tenderness, and swelling of the left eye. A surgical incision was noted on the left orbital region. Left eye movements were restricted.. Immunohistochemical examination revealed pan-cytokeratin (PCK, +), p63 (partial, +), cytokeratin 7 (CK7, +), cytokeratin 14 (CK14, +), epithelial membrane antigen (EMA, +), protein expressed by erythroblast transformation-specific related gene (ERG, -), S-100 (, -), Epstein-Barr virus-encoded small RNA (EBER, -), smooth muscle actin (SMA, -), and Ki-67 (with a proliferation index approximately 40%). After carefully reviewed the manifestations, imaging findings, and immunohistochemical evidences, a diagnosis of poorly differentiated adenocarcinoma of lacrimal gland was made.. Based on the gene sequencing results, we started the patient with an intensive PF chemotherapy including a combination of cisplatine, fluorouracil, and epirubicin. Two months later, radiotherapy was introduced to the therapy regimen.. The patient responded well to the treatment without severe adverse events. MRI scan showed remarkable remission.. This rare case report will help raise the awareness of high grade lacrimal gland cancer, and subsequently aid the diagnosis in future cases. Positive immunohistochemical markers of CK7, CK14, EMA, p63, and high proliferation index of Ki-67 can help establishing a diagnosis, and cisplatine-fluorouracil program is proved feasible. We share the difficulties we have encountered, hoping to improve patient care in the future.

Topics: Adenocarcinoma; Aged; Antineoplastic Combined Chemotherapy Protocols; Biomarkers, Tumor; Eye Neoplasms; Humans; Keratins; Lacrimal Apparatus Diseases; Male

To report a case of caruncular dacryops in a 58-year-old man that was excised in its entirety and to offer an immunohistopathologic analysis.. Sections stained with hematoxylin and eosin, periodic acid-Schiff, and Grocott methenamine silver (the latter 2 for identification of mucus) were evaluated, and immunohistochemical investigations were performed using cytokeratin (CK) 7, CK14, CK17, and smooth muscle actin.. Histopathologic examination revealed a cystic dilation of the lacrimal gland ducts containing secretory globules. The ducts were composed of double-layered cuboidal epithelium with rare scattered goblet cells and interspersed prominent lobules of lacrimal gland tissue, diagnostic of dacryops. Immunohistochemistry of cystic ducts demonstrated a CK profile identical to that of the conjunctiva including the absence of a myoepithelium.. This is the first case of an intact caruncular lacrimal ductal cyst (dacryops). A previous report documented a spontaneously collapsed cyst with extrusion of secretory globoid bodies into extracellular space that elicited a foreign body giant cell response.

Topics: Actins; Conjunctiva; Cysts; Goblet Cells; Humans; Immunohistochemistry; Keratins; Lacrimal Apparatus Diseases; Male; Middle Aged; Staining and Labeling

Basal cell adenocarcinoma is a recently recognized entity among malignant tumors of the salivary glands. This entity has not received enough attention among ophthalmologists and ophthalmic pathologists. We recommend that basal cell adenocarcinoma be included in the differential diagnosis of lacrimal gland tumors.. Case report.. In 1988 a lacrimal gland tumor was incompletely removed from the left orbit of a 36-year-old woman and diagnosed as "solid basaloid adenoid cystic carcinoma". Soon after, an exenteration of the orbit was performed. In 1998, upon review of the initial histopathology, the diagnosis of solid adenoid cystic carcinoma was changed to basal cell adenocarcinoma. RESULTS AND MAIN OUTCOME MEASURE: The pathologic findings included nests of basaloid cells with minimal atypia and incomplete palisading around the periphery. The cells were of two types. The first type were large, pale cells with round or oval nuclei, scanty cytoplasm, and ill-defined borders. The other type were smaller cells with hyperchromatic nuclei situated mainly near the periphery of the nests. There was no necrosis or perineural invasion. Mitotic figures were present. Cysts within the nests showed Alcian blue negative contents. Immunohistochemistry showed a positive reaction to cytokeratin and a negative reaction with smooth muscle actin (SMA).. The Alcian blue negative stain, the negative reaction to SMA, and the fact that the patient is still alive 10 years after exenteration favored the diagnosis of basal cell adenocarcinoma. It is essential to differentiate a basal cell adenocarcinoma of the lacrimal gland from the solid basaloid type of adenoid cystic carcinoma, because the former has a better prognosis. Patients with basal cell adenocarcinomas of the lacrimal gland should be closely monitored for local recurrences because this tumor has the tendency to show perineural invasion.

Topics: Adenocarcinoma; Adult; Biomarkers, Tumor; Diagnosis, Differential; Eye Neoplasms; Female; Humans; Immunoenzyme Techniques; Keratins; Lacrimal Apparatus Diseases; Mucin-1; S100 Proteins; Tomography, X-Ray Computed

Tumor cells from a malignant mixed tumor of the lacrimal gland were maintained in tissue culture for more than 55 generations. Comparative immunohistochemical analysis was performed on whole tumor sections and on the tumor cell culture to define the origin of the cells in culture. The cultured cells expressed cytokeratin, smooth-muscle actin, S-100 protein, and vimentin and were negative for glial fibrillary acidic protein. Tumor sections expressed cytokeratin but were negative for muscle-specific actin, vimentin, and glial fibrillary acidic protein. Through tissue culture studies of salivary gland epithelial neoplasias, which are very similar to lacrimal gland epithelial neoplasias, pluripotential stem cells have been identified. Similar tissue culture analysis of lacrimal gland epithelial neoplasms can be a valuable tool for studying the origin of these uncommon tumors.

Topics: Antigens, Neoplasm; Biomarkers, Tumor; Chromosome Aberrations; Chromosome Disorders; Eye Neoplasms; Humans; Immunohistochemistry; Karyotyping; Keratins; Lacrimal Apparatus; Lacrimal Apparatus Diseases; Male; Mixed Tumor, Malignant; Tumor Cells, Cultured

A right orbital tumor was excised from a 76-year-old woman. Pathological examination showed that the tumor was composed of spindle to cuboidal cells arranged in a solid to trabecular pattern. Immunohistochemical stains were positive for S-100 protein, muscle-specific actin, cytokeratins MAK6 and AE1,3, and glial fibrillary acid protein and negative for CD34 in tumor cells. Ultrastructural features of tumor cells included microvillous processes, intercellular junctions, and intracytoplasmic filaments with electron densities. To our knowledge, this is the first non-spindle cell myoepithelioma noted to arise in the lacrimal gland. This tumor likely has a similar biological behavior to pleomorphic adenoma (benign mixed tumor).

Topics: Actins; Aged; Female; Glial Fibrillary Acidic Protein; Humans; Immunohistochemistry; Keratins; Lacrimal Apparatus Diseases; Myoepithelioma; S100 Proteins

To their knowledge, the authors report the first recognized case of ductal adenocarcinoma of the lacrimal gland (histologic equivalent of salivary duct carcinoma). Primary adenocarcinoma of the lacrimal gland is rare and has been described generically. In contrast, primary adenocarcinomas of the major and minor salivary glands are much more common and have been classified into histopathologic subtypes that have different clinical characteristics and outcomes.. A 68-year-old man presented with a 6-month history of a painless mass in the right upper outer eyelid. The authors describe the clinical, radiologic, and histopathologic features of this case and review the lacrimal gland literature.. A modified en bloc orbitectomy was performed, and postoperative radiotherapy was administered. The patient was alive and well without evidence of tumor recurrence 10 months after surgery.. The World Health Organization classification of salivary adenocarcinomas (1991) provides a framework for further insight into the presentation and biologic behavior of the less common lacrimal carcinomas.

Topics: Aged; Carcinoma, Ductal, Breast; Humans; Immunoenzyme Techniques; Keratins; Lacrimal Apparatus Diseases; Male; Orbit; Radiotherapy, Adjuvant; Salivary Ducts; Salivary Gland Neoplasms; Tomography, X-Ray Computed

Solitary fibrous tumors are rare spindle cell neoplasms that most commonly arise in the pleura. Recently, solitary fibrous tumors of the orbital soft tissues have been described. The authors report the first case of solitary fibrous tumor arising in the lacrimal gland fossa and discuss features that may distinguish solitary fibrous tumor from other lacrimal fossa lesions.. A left lacrimal gland fossa tumor was excised via lateral orbitotomy from a 76-year-old man and analyzed by light microscopy. Immunohistochemical studies were performed using antibodies to CD34, vimentin, epithelial membrane antigen, cytokeratins AE1-3 and Cam5.2, S-100 protein, and actin.. The histopathologic features of the tumor were typical of solitary fibrous tumor. A well-circumscribed, variably cellular proliferation of bland spindle cells displayed both "patternless" and storiform growth patterns. Immunohistochemical studies showed that the tumor cells expressed vimentin and CD34 but did not express epithelial membrane antigen, cytokeratins, S-100 protein, or actin. The patient is free of tumor recurrence after 1 year of follow-up.. The authors report the first case of solitary fibrous tumor arising in the lacrimal gland fossa. The clinical presentation of this tumor was similar to that of pleomorphic adenoma (benign mixed tumor) of the lacrimal gland; fortunately, the treatment for both of these lacrimal gland fossa neoplasms is en bloc excision. The histopathologic appearance of solitary fibrous tumor is varied, and immunohistochemical analysis may help in distinguishing solitary fibrous tumor from other lacrimal fossa lesions. Careful follow-up of patients with solitary fibrous tumor is important because recurrence may present years after excision of the primary tumor.

Topics: Aged; Antigens, CD34; Biomarkers, Tumor; Fibroma; Humans; Immunohistochemistry; Keratins; Lacrimal Apparatus Diseases; Male; Mucin-1; S100 Proteins; Tomography, X-Ray Computed; Vimentin

The triple A or Allgrove's syndrome (MIM*231550) is an autosomal recessive disease characterized by the triad of adrenocorticotropic hormone (ACTH) resistant adrenal insufficiency, achalasia and alacrima. Since its first description by Allgrove et al. (1978) more than 70 cases from all over the world have been reported. The syndrome manifests itself during the first decade of life with severe hypoglycaemic episodes which can cause sudden death. The frequent association with neurological disorders presenting as a mixed pattern of upper and lower motor neuropathy, sensory impairment, autonomic neuropathy and mental retardation may result in a severely disabling disease. As an additional feature some patients have hyperkeratosis of their palms and soles. We have performed a systematic genome linkage scan in eight triple A families of which three were consanguineous [including the large highly inbred kindred described by Moore et al. (1991)]. We obtained conclusive evidence for linkage of the triple A syndrome locus to markers on chromosome 12q13 (D12S368, theta max = 0, Zmax = 10.81) with no indication of genetic heterogeneity. Haplotype and multipoint analyses suggest that the gene is located on a chromosomal segment flanked by the markers D12S1629 and D12S312 which are 6 cM apart. This region harbors the type II keratin gene cluster, and potential candidate genes include SCN8A and HOXC genes.

Topics: Adrenal Insufficiency; Chromosome Mapping; Chromosomes, Human, Pair 12; Esophageal Achalasia; Female; Genetic Linkage; Humans; Keratins; Lacrimal Apparatus Diseases; Male; Multigene Family; Pedigree; Syndrome

An 18-year-old woman underwent exenteration of the right orbit for tumor recurrence 3 years subsequent to external-beam irradiation for a lacrimal gland tumor diagnosed as an "adenocarcinoma." Light microscopy of the exenteration specimen revealed an acinic cell carcinoma of the lacrimal gland, with a predominant microcystic (latticelike) pattern of growth. Cytoplasmic vacuoles and the secretion within the microcysts stained positive with periodic acid-Schiff with and without alpha-amylase, alcian blue (at a pH of 2.5), mucicarmine, and colloidal iron with and without hyaluronidase. This histochemical staining for epithelial mucins supports the theory that the lacrimal gland, although serous in type, may also function as a modified mucus gland. There was cytoplasmic immunopositivity for keratin (CAM 5.2, KAE 1-3); immunostaining for vasoactive intestinal polypeptide was negative. Electron microscopy disclosed undifferentiated features of intercalated duct cells. We speculate that the lack of immunoreactivity for vasoactive intestinal polypeptide may be correlated with the predominantly undifferentiated intercalated duct cell features observed ultrastructurally.

Topics: Adolescent; Carcinoma, Acinar Cell; Cobalt Radioisotopes; Female; Histocytochemistry; Humans; Immunohistochemistry; Keratins; Lacrimal Apparatus Diseases; Mucins; Neoplasm Recurrence, Local; Orbit; Tomography, X-Ray Computed; Vasoactive Intestinal Peptide

A 50-year-old man presented with a rapidly growing mass in the area of the right lacrimal gland. An initial erroneous histopathologic diagnosis of a pleomorphic adenoma made on a small-incisional biopsy was later corrected to a malignant rhabdoid tumor when a wide local excision of the tumor was performed. The tumor was composed predominantly of dyscohesive, globoid, and eosinophilic cells, which frequently contained cytoplasmic inclusions. These were demonstrated to be composed of whorls of intermediate vimentin filaments. The tumor cells expressed epithelial membrane antigen as well as cytokeratin. Ultrastructurally, they displayed intercellular junctions and interrupted segments of linear basement membrane material. These findings, together with the development of the lesion within the parenchyma of the lacrimal gland, are suggestive of an epithelial origin. The patient was treated with radical surgery and adjunctive radiotherapy and chemotherapy, which are the recommended treatment modalities for this highly malignant tumor.

Topics: Antigens, Neoplasm; Antineoplastic Combined Chemotherapy Protocols; Combined Modality Therapy; Eye Neoplasms; Humans; Keratins; Kidney Neoplasms; Lacrimal Apparatus Diseases; Male; Middle Aged; Rhabdomyoma

34 cases of lacrimal epithelial tumor were studied with the anti-keratin and anti-CEA monoclonal antibody peroxidase-anti-peroxidase staining and the AB/PAS mucohistochemical staining. It was found that HK2 was positive in the gland-luminal cells and cancer cells in the squamous metaplastic portion of the tumor; K12 was positive in the mucoid, fibroid, and cartilaginoid metaplastic tumor cells, while K27 was positive in less number of the squamous cells; CEA was positive in tumor cells of the nest and glandular lumina. Mecohistochemically, the tumor cells were 100% positive. The authors were of the opinion that keratin HK2 and K12 and CEA were ideal markers for lacrimal epithelial tumors.

Topics: Antibodies, Monoclonal; Biomarkers, Tumor; Carcinoembryonic Antigen; Cystadenocarcinoma; Eye Neoplasms; Humans; Immunohistochemistry; Keratins; Lacrimal Apparatus Diseases; Neoplasms, Germ Cell and Embryonal

The distribution of cytokeratins (CK), actin, lactoferrin (Lf), lysozyme (Ly), vimentin and S-100 protein was immunohistochemically investigated in paraffin-embedded specimens of five inflammatory and five neoplastic lesions of lacrimal glands (LGs). Atrophic acini in dacryoadenitis reacted with antibodies (ABs) KL1 and Pkk1 (CK 7, 8, 17, 18) in a manner similar to ducts. Apart from myoepithelial cells and some luminal-duct cells, the remaining epithelia in dacryoadenitis were negative with AB 34 beta E12 (CK 5). The number of AB HHF35 (actin)-positive myoepithelial cells was not altered in dacryoadenitis. Epithelia in dacryoadenitis reacted weakly but consistently with Lf while revealing weak and inconsistent staining for Ly. Vimentin was negative in epithelial cells in dacryoadenitis except in one case. S-100 protein was detected only in epithelia of inflammatory major LGs. Epimyoepithelial islands in lymphoepithelial proliferation reacted variably for CKs, Lf, Ly and vimentin and remained negative for actin and S-100. In pleomorphic adenomas, neoplastic cells showing duct-like differentiation (luminal) reacted consistently with CK 7, 8, 17, 18 and S-100 protein and inconsistently with CK 5, Lf and Ly but remained negative for actin and vimentin. Other neoplastic cells (ovoid/peripheral cells) stained consistently for CK 5, vimentin and S-100 protein and focally for CK 7, 8, 17, 18, actin, Lf and Ly. Spindle-form neoplastic cells found in the stroma exhibited vimentin and S-100 protein and, less frequently, actin. Determination of these antigens in pleomorphic LG adenomas may help to evaluate their prognosis.

Topics: Actins; Biomarkers; Dacryocystitis; Eye Neoplasms; Female; Humans; Immunoenzyme Techniques; Infant; Keratins; Lacrimal Apparatus; Lacrimal Apparatus Diseases; Lactoferrin; Male; Middle Aged; Muramidase; S100 Proteins; Vimentin

To determine whether there are specific cytologic features associated with primary Sjögren's syndrome (SS), the authors evaluated impression cytology specimens from three conjunctival sites (temporal bulbar [TB], inferior bulbar [IB], and inferior tarsal [IT]) from 38 SS eyes, 34 eyes of aqueous tear-deficient patients without SS, 35 eyes of seborrheic blepharitis patients, and 17 eyes of normal controls in a masked fashion. The following features were observed more frequently in SS eyes than in the eyes of the other groups: squamous metaplasia of the TB and IB (P less than 0.05), extensive (greater than 75%) goblet cell loss of the TB (P less than 0.05), mucous aggregates of the bulbar conjunctiva (P less than 0.05), and inflammatory cells intercalated with epithelial cells on the IT conjunctiva (P less than 0.06). The conjunctival inflammatory cell infiltrate correlated with the presence of extensive squamous metaplasia (P less than 0.01) in SS specimens. The inflammatory cells on the IT conjunctival epithelium were found to consist predominantly of T-lymphocytes by immunofluorescent staining of cytologic specimens from six eyes. Based on these findings, the authors speculated that conjunctival squamous metaplasia, in addition to aqueous tear deficiency, may be due to primary involvement of the dysfunctional immune system of SS.

Topics: Antibodies, Monoclonal; Antigens, CD; Antigens, Differentiation, B-Lymphocyte; Antigens, Differentiation, T-Lymphocyte; Blepharitis; CD3 Complex; Cell Adhesion Molecules; Chi-Square Distribution; Conjunctiva; Epithelium; Humans; Immunoenzyme Techniques; Keratins; Lacrimal Apparatus Diseases; Lectins; Receptors, Antigen, T-Cell; Reproducibility of Results; Sialic Acid Binding Ig-like Lectin 2; Sjogren's Syndrome; T-Lymphocytes

We studied the immunohistopathologic features of normal lacrimal gland, benign mixed tumor, and malignant mixed tumor of the lacrimal gland. Primary antisera were to keratin, muscle-specific actin, vimentin, and glial fibrillary acid protein. Keratin stained in occasional myoepithelial cells in normal gland, ductal epithelium in normal gland and the tumors, and occasional stromal epithelioid cells in the tumors. Muscle-specific actin stained in myoepithelium and vascular smooth muscle in normal gland and the tumors, and occasional spindle-shaped and clusters of stromal cells in the tumors. Vimentin staining was nonspecific. Glial fibrillary acid protein stained in occasional myoepithelial cells in normal gland and polyhedral stromal cells in benign mixed tumor. Our findings indicate that ductal epithelium develops into the epithelial component, and some cells in the stroma and myoepithelium develop into some cells in the stroma of benign and malignant mixed tumor of the lacrimal gland.

Topics: Actins; Adolescent; Adult; Aged; Epithelium; Eye Neoplasms; Female; Glial Fibrillary Acidic Protein; Humans; Immunoenzyme Techniques; Keratins; Lacrimal Apparatus; Lacrimal Apparatus Diseases; Male; Middle Aged; Neoplasms, Germ Cell and Embryonal; Vimentin

Ten malignant myoepithelial tumors of the salivary glands and one of lacrimal gland origin were studied by light, electron microscopy and immunocytochemistry. The light microscopic appearance of the tumors varied from primarily spindle cell neoplasms (two cases), to others with predominantly epithelial components (four cases) and mixed varieties (five cases). Therefore, they can be confused with other epithelial and mesenchymal neoplasms. The electron microscopic spectrum varied from tumors with widespread and typical myoepithelial differentiation (i.e. myofilament bundles at the cell periphery, attachment plaques and intercellular junctions) to some with diffusely distributed filaments, without associated spindle densities but with attachment plaques, and others with evidence of duct formation and containing scattered cells showing intracytoplasmic tonofilaments. Often the tumors revealed mixed ultrastructural features; the relative numbers of the different cellular components was variable. The eleven neoplasms were S-100 protein, actin and keratin positive, either focally or diffusely, with varying degrees of intensity. Ten of the eleven tumors were positive for vimentin and nine of ten tested expressed carcinoembryonic antigen. Only two of nine were focally positive for glial fibrillary acidic protein. The study emphasizes the variable light microscopic appearances of these neoplasms and their immunocytochemical and ultrastructural spectrum. Accurate determination of myoepithelial differentiation sometimes requires careful evaluation of the light, ultrastructural and immunocytochemical findings. If all three diagnostic modalities are not utilized, it is likely that some of these neoplasms will be improperly classified.

Topics: Actin Cytoskeleton; Actins; Adenoma, Pleomorphic; Adult; Aged; Carcinoembryonic Antigen; Female; Glial Fibrillary Acidic Protein; Humans; Immunohistochemistry; Keratins; Lacrimal Apparatus; Lacrimal Apparatus Diseases; Male; Microscopy, Electron; Middle Aged; Myoepithelioma; Neoplasms, Germ Cell and Embryonal; S100 Proteins; Salivary Gland Neoplasms; Vimentin

Dacryoliths are uncommon and there are only a few reports in the literature. In a patient with recurrent stenosis of the lacrimal drainage system a lacrimal stone (dacryolith) was removed from the lacrimal sac during dacryocystorhinostomy. A histological and immunohistochemical examination was performed and keratin antibodies could be detected. In a chemical analysis calcium, sulfur and some phosphorus were determined as major constituents.

Topics: Adult; Antibodies; Calculi; Female; Humans; Keratins; Lacrimal Apparatus Diseases