Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UNIPROT:O76050 (neu)
 3,969 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Primary signet ring cell carcinoma of the eyelid is a rare neoplasm with less than ten cases described. This report details another case, which shows further parallels to lobular carcinoma of the breast, and reviews the literature on this subject. A 73-year-old white female presented with diffuse induration of her left eyelids. Histopathology revealed a delicate infiltrate of epithelial cells scattered throughout the lid stroma in a non-destructive pattern. The cells were relatively monomorphous and showed only mild atypia and rare mitotic figures. Many had slightly granular amphophilic cytoplasms, others showed distinct signet ring cell morphology, and all were strongly positive for epithelial mucin. Immunomicroscopy showed strong reactivity for estrogen receptor (ER), progesterone receptor (PR) and gross cystic disease fluid protein-15 (GCDFP-15), and was negative for Her-2/neu (erb-2) and cytokeratin 20. An extensive workup for other primary sites was negative. Orbital exenteration showed extensive involvement of both lids and soft tissue, including diffuse muscle and lacrimal gland infiltration. In the breast, signet ring cell carcinoma is considered a variant of lobular carcinoma. The delicate infiltrating pattern in our case and the ER+, PR+, GCDFP-15+, Her-2/neu-phenotype further strengthen this analogy. Together, these data also support apocrine differentiation of primary eyelid signet ring cell carcinoma.
 ...
PMID:Primary signet ring cell carcinoma of the eyelid: report of a case demonstrating further analogy to lobular carcinoma of the breast with a literature review. 1180 78

A 78-year-old male patient presented with double vision, painless palpable mass under the right superolateral orbital rim, downward displacement and restricted adduction of the right eye. His visual acuity was 20/50 OD and 20/20 OS. Hertel exophthalmometry was 21 mm OD and 17 mm OS. Computed tomographic scans showed an infiltrative orbital mass with ill-defined, irregular margins, involving the lacrimal gland and the lateral rectus muscle. The patient underwent an anterior transcutaneous transseptal orbitotomy with incisional biopsy and surgical debulking. Histopathologic evaluation revealed primary ductal adenocarcinoma of the lacrimal gland. Following the metastatic work up, he underwent an eyelid-sparing orbital exenteration. Microscopically, the tumor elements were characterized by large polygonal cells with vesicular nuclei, prominent nucleoli and amphophilic cytoplasm. The tumor components comprised duct-type structures with papillary and cribriform patterns, surrounded by prominent basement membrane. The tumor cells were positive for cytokeratin-7, matrix metalloproteinase (MMP)-2, MMP-9, MMP-13 and proto-oncogene Her-2/neu, but negative for cytokeratin-5, cytokeratin-20, p63, prostate-specific antigen, S-100 protein and thyroid transcription factor. These histopathologic findings were compatible with poorly differentiated ductal adenocarcinoma of the lacrimal gland, T3N0M0. Twenty-four months after orbital exenteration, the patient was diagnosed with ipsilateral parotid gland and cervical lymph node metastases and died of disease.
 ...
PMID:Primary ductal adenocarcinoma of the lacrimal gland: case report. 2255 22

Carcinoma of the lacrimal gland is a rare malignancy, limiting opportunities to develop new therapeutic regimens through clinical trials. There are no standard guidelines on optimal treatment of lacrimal gland carcinoma. In addition, lacrimal gland carcinoma includes several different subtypes with distinct behavior and response to treatment, further complicating treatment. Overexpression of the Her-2/neu protein, a potential target for new therapeutic agents, has previously been described in lacrimal gland carcinoma; however, there are no published reports regarding treatment of lacrimal gland cancer with Her-2 directed medications. This case report describes treatment of a patient with metastatic lacrimal gland carcinoma with lapatinib, an oral agent with activity against Her-2/neu amplified malignancies. In this case, Her-2 overexpression was confirmed by biopsy of a metastatic site. PET imaging obtained 6 months after the initiation of lapatinib showed evidence of a partial response to treatment, although the patient later developed progressive neurologic complications related to her malignancy and ultimately died. Lapatinib and other Her-2 targeted agents may represent an effective therapeutic option for this rare malignancy, in cases of metastatic disease.
...
PMID:Metastatic, her-2 amplified lacrimal gland carcinoma with response to lapatinib treatment. 2572 2

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.
 ...
PMID:Adenocarcinoma, Not Otherwise Specified, Arising in Accessory Lacrimal Gland: A Diagnostic Challenge. 3032 83