Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0677930 (primary tumor)
 20,210 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two hundred and twenty-seven cases of carcinoma metastatic to the eye and orbit have been reviewed previously. The orbit alone was involved in 28 cases: theses lesions constitute the basis of this clinicopathologic study. The most common signs and symptoms produced by orbital metastasis included exophthalmos (75%1, pain (29%), decreased vision (29%), periorbital swelling (25%), a visible mass (21%), ophthalmoplegia, and diplopia (18%). The ophthalmologists' preoperative (or premortem) clinical diagnoses were: orbital mass, 36%; metastatic carcinoma, 29%; leukemia, 7%; melanosarcoma, 4%; mixed tumor of lacrimal gland, 4%; and meningioma, 4%. The sites of the primary tumors in the 28 patients with orbital metastasis were as follows: breast, eight; lung, four; genitourinary tract, four; pancreas, one; and ileum, one. In 10 patients the site of the primary carcinoma was not determined. In 17 of the 28 patients, symptoms of orbital metastasis preceded detection of a primary tumor elsewhere in the body. In 10 of the remaining 11 patients, detection of the primary tumor had preceded the onset of orbital symptoms. In one patient, symptoms of the primary tumor and of orbital metastasis appeared at about the same time. The median survival of patients with carcinoma metastatic to the orbit was 15.6 months from the time of orbital surgery. This was much better than the median survival of the 227 patients in the overall study (7.4 months) and far better than the median survival of the patients with metastasis to the anterior segment of the eye (only 5.4 months).
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PMID:Carcinoma metastatic to the eye and orbit III. A clinicopathologic study of 28 cases metastatic to the orbit. 95 71

In 16 patients the histologically proven diagnosis of a lacrimal sac tumor was made; in 9 of them a paranasal sinus tumor had invaded the lacrimal pathways, in 7 patients existed a primary tumor of the lacrimal sac, A- and B-scan sonography allow a clear-cut differentiation of both these entities. Furthermore primary and tumor-related inflammations of the lacrimal pathways can be distinguished by echography, which thereby provides the decisive diagnostic basis for the different therapeutic procedures in each case.
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PMID:[Echographic diagnosis in tumors of the paranasal sinuses with invasion of the lacrimal ducts]. 332 Jun 44

In this report a patient with a lymphoepithelioma (LE)-like carcinoma of the lacrimal gland is described for the first time in the literature. LE-like carcinomas outside the nasopharynx rarely occur in the major and minor lacrimal glands of natives of Greenland, Inuit or natives of southern China. The patient's tumor was extirpated using a Kronlein approach followed by total parotidectomy and modified radical neck dissection on the ipsilateral side after the detection of suspicious lymph nodes by ultrasound transmission. Adjuvant radiochemotherapy with cisplatin and 5-fluorouracil was then carried out. Three years later there is no sign of recurrence. As a result of this case we recommend careful examination of the orbit and lacrimal gland in cases of LE-like cancer with an unidentified primary tumor.
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PMID:Lymphepithelioma-like carcinoma of the lacrimal gland. 1105 55

Primary sebaceous carcinoma is an exceptionally rare tumor of the lacrimal gland and less than 10 cases have been so far published in the literature. Two adult patients aged 38 and 81 years, respectively, who suffered unilateral painful massive swelling of the lacrimal gland are described. The disease in the first patient initially manifested as ipsilateral parotid gland metastasis and the primary tumor could be detected 3 months later. Both tumors were rock hard and fixed on palpation, caused partial upper eyelid ptosis, displaced the globe anteromedially and impaired ocular motility. Magnetic resonance imaging studies showed mostly homogeneous, well-delineated and moderately contrast-enhancing lacrimal gland fossa tumors without bone destruction. The management consisted of incisional biopsy for the diagnosis, immediately followed by exenteration. The younger patient further underwent radical neck dissection, parotidectomy and orbital and neck radiotherapy, which provided him a 2-year disease-free survival. Histopathological examination showed poorly differentiated sebaceous carcinoma destructing completely the lacrimal gland with predominantly comedo pattern. Despite its rarity and lack of specific clinical and imaging signs, sebaceous carcinoma should be considered in rapidly evolving painful and hard lacrimal gland fossa tumors. Also noteworthy is the early propensity of this tumor to spread to regional draining lymph nodes and the parotid gland in particular.
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PMID:Primary sebaceous carcinoma of the lacrimal gland. 2290 82

A majority of intraocular tumors can be diagnosed based on clinical examination and ocular imaging studies, which obviate the need for diagnostic ophthalmic fine needle aspiration biopsy (FNAB). Overall, diagnostic accuracy of ophthalmic FNAB is high but limited cellularity can compromise the diagnostic potential of ophthalmic aspirate samples. The role of ophthalmic FNAB is limited in retinal tumors. Orbital FNAB should be considered in the evaluation of lacrimal gland tumors, orbital metastasis, and lymphoproliferative lesions. Negative cytologic diagnosis of malignancy should not be considered unequivocal proof that an intraocular malignancy does not exist. With improved understanding of genetic prognostic factors of uveal melanoma, ophthalmic FNAB is gaining popularity for prognostic purposes in combination with eye conserving treatment of the primary tumor. In special clinical indications, ancillary studies such as immunohistochemistry and FISH can be performed on ophthalmic FNAB samples. Assistance of an experienced cytopathologist cannot be overemphasized.
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PMID:Fine needle aspiration biopsy of ophthalmic tumors. 2396 Sep 81

Renal carcinoid tumor is an exceedingly rare malignancy. A 57-year-old man with a renal carcinoid tumor discovered after metastasizing to intraocular and bilateral orbital structures is described. The patient presented with a blind painful OS and a right superotemporal subconjunctival mass. Imaging studies revealed a large left intraocular tumor, a mass in the left medial rectus muscle, and right lacrimal gland enlargement. The OS was enucleated, and incisional biopsies were performed from the other 2 lesions. Histopathological studies demonstrated metastatic neuroendocrine tumor with chromogranin and synaptophysin positivity. Systemic work up revealed a right renal mass and multiple hepatic metastatic lesions. Radical nephrectomy was performed, and octreotide, capecitabine, and temozolomide were administered. Removal of the primary tumor and the eye that had no prospect for useful vision and further treatment with octreotide, capecitabine, and temozolomide provided a disease progression-free period of 24 months and allowed the patient to function normally.
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PMID:Renal Carcinoid Tumor Metastatic to the Uvea, Medial Rectus Muscle, and the Contralateral Lacrimal Gland. 2477 67

Adenoid cystic carcinoma (ACC) can arise in several organs, and prognosis is highly dependent on the primary tumor site. Primary cutaneous ACC has an excellent prognosis compared with salivary or lacrimal ACC. Activation of MYB by gene fusion or other mechanisms has been found in salivary, breast, and lacrimal ACCs but has not been described in cutaneous ACC. We analyzed the histopathologic and immunohistochemical features of 19 primary cutaneous ACCs, 2 periorbital ACCs, and 12 salivary gland ACCs and assessed for MYB activation in primary cutaneous ACC by immunohistochemistry and molecular methods. The presence of perineural invasion differed significantly among ACCs of various sites (83% salivary, 50% eyelid, 11% skin, P=0.0002). Over 90% of all ACCs were grade 1 or 2 and exhibited diffuse (>50%) positivity with CD117, SOX-10, and smooth muscle actin immunostains. CK15 and vimentin showed diffuse positivity in 36% and 57% of cutaneous ACCs, respectively, and were negative or only focally positive in all salivary ACCs (P=0.04 and 0.002). Six of the 11 cutaneous and periorbital ACCs tested with reverse transcriptase polymerase chain reaction and/or fluorescence in situ hybridization had MYB rearrangements including 2 cases that expressed MYB-NFIB fusion transcripts. Diffuse expression of MYB protein assessed by immunostaining was present in 8 of 9 cutaneous ACCs, including cases both with and without MYB rearrangements. These results indicate that cutaneous ACCs possess the same types of MYB alterations as ACCs of other anatomic sites. Vimentin and CK15 appear to have some discriminatory value in differentiating between primary cutaneous and salivary gland ACCs.
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PMID:Detection of MYB Alterations and Other Immunohistochemical Markers in Primary Cutaneous Adenoid Cystic Carcinoma. 2607 64

A 40-year-old man presented with right eye axial proptosis and ophthalmoplegia for 3 months. Imaging study showed a right intraconal mass with the erosion of the orbital floor. Incisional biopsy revealed mucoepidermoid carcinoma. Nasal endoscopy was normal and systemic tumor screening was negative for a primary source. The patient underwent right orbital exenteration, uncinectomy, nasal and maxillary mucosal biopsy. Malignant cells were found present in the mucosa of maxillary sinus roof and uncinate bone. The postoperative positron emission tomography scan showed residual active lesion in right orbital apex and maxilla but no primary lesion elsewhere. The patient subsequently underwent 35 cycles of postoperative radiotherapy. Primary mucoepidermoid carcinoma of the orbit is rare and typically arises from the lacrimal gland or sac. Those tumors not arising from lacrimal apparatus should be presumed metastatic in origin, and the thorough systemic survey should be undertaken in the search for the primary tumor.
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PMID:Secondary mucoepidermoid carcinoma of the orbit. 2714 39

Neoplasms arising in accessory lacrimal glands are rare. We describe a 33-year-old man with adenocarcinoma arising in the left lower eyelid accessory lacrimal gland. Microscopic evaluation demonstrated an infiltrative neoplasm composed of mildly to moderately pleomorphic cells with abundant eosinophilic cytoplasm and focal intracytoplasmic vacuoles, arranged predominantly in ductules. Foci of luminal and intracytoplasmic eosinophilic secretory material and occasional mucin were noted. An in situ component was identified in the gland of Wolfring. Though perineural invasion was present, high-grade nuclear features, brisk mitotic activity, and comedonecrosis were not identified. Immunohistochemical studies were notable for immunoreactivity of the tumor cells for CK7, carcinoembryonic antigen, BRST-2, androgen receptors, and HER2/neu (2+). The neoplastic cells were negative for CK20, estrogen and progesterone receptors, S-100, p63, calponin, thyroid transcription factor-1, and prostate-specific antigen. Fluorescence in situ hybridization studies for ETV6 and MAML2 rearrangements and for HER2/neu amplification were negative. Because of the absence of unifying morphologic, immunophenotypic, and molecular genetic findings, the diagnosis of adenocarcinoma, not otherwise specified, was rendered. The patient underwent comprehensive oncologic workup, which was negative for another primary tumor and metastases. He remains disease free with a follow-up of 4 years. This case illustrates the challenges encountered in applying salivary gland tumor classification to the accessory lacrimal gland neoplasm.
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PMID:Adenocarcinoma, Not Otherwise Specified, Arising in Accessory Lacrimal Gland: A Diagnostic Challenge. 3032 83

The eye is a rare site for disseminated malignancies; nevertheless, several tumors may metastasize to ocular structures. Few cases of urothelial and bladder cancer with eye involvement have been described in the literature thus far. The rarity of metastatic ocular localization implies an accurate differential diagnosis among the possible primary tumor sites. However, a specific diagnostic algorithm is not currently available, nor a defined therapeutic approach. Eye metastases are associated with advanced disease and poor prognosis. Physicians should be made aware of the possibility of eye involvement in patients with a past medical history of urothelial bladder cancer associated with ocular symptoms. The present case reports discusses the first documented case, to the best of our knowledge, of an urothelial bladder cancer metastasizing to the retro bulbar region that infiltrates the lacrimal gland. Furthermore, the report provides a systematic qualitative review of the current literature on eye metastases from urothelial bladder cancer using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses.
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PMID:Urothelial bladder carcinoma metastasizing to the eye: A systematic review and case report. 3065 88


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