Angiosarcoma of the eyelid: a clinicopathologic comparison between isolated unilateral tumors and tumors demonstrating extrapalpebral involvement.

Angiosarcomas involving the head and neck are malignant tumors which tend to involve multiple anatomical structures with an overall dismal prognosis. Reports of primary, isolated eyelid involvement are rare. We report 4 cases of angiosarcoma involving the eyelid as either an isolated tumor or as part of a more diffuse malignant process and compare the features of these 2 tumor types to cases reported in the literature. The mean age at which patients develop angiosarcoma involving the eyelid was 72. Patients with isolated tumors most often presented complaining of a discrete mass clinically resembling a stye. Patients with isolated eyelid involvement had better survival (100% at 3.2 years) compared with patients with diffuse disease (57% at 3.3 years). Those with isolated eyelid involvement have an average tumor size of 2.08 cm with no distant metastasis. Patients who have eyelid angiosarcoma with extrapalpebral involvement have tumors ranging between 5 and 10 cm on average and 21% develop metastases. True isolated angiosarcoma of the eyelid is rare. When extrapalpebral involvement is ruled out by clinical and radiographic examination, patients with isolated involvement seem to fare better compared with patients with eyelid involvement secondary to more regionally extensive tumors.

Rare double-hit with two translocations involving IGH both, with BCL2 and BCL3, in a monoclonal B-cell lymphoma/leukemia.

BACKGROUND: Chronic Lymphocytic Leukemia (CLL) is a lymphoproliferative disease characterized by multiple recurring clonal cytogenetic anomalies and is the most common leukemia in adults. Chromosomal abnormalities associated with CLL include trisomy 12 and IGH;BCL3 rearrangement [t(14;19)(q32;q13)] that juxtaposes a proto-oncogenic gene BCL3 and an immunoglobulin heavy chain, a translocation that may be associated with shorter survival. In addition to the IGH;BCL3 rearrangement, other translocations involving 14q32 locus are involved in various lymphoproliferative pathologies pointing toward the significance of IGH locus in oncogenic progression. Significantly, in the majority of B-cell neoplasms that carry an IGH;BCL3 rearrangement, it is a sole translocation involving an IGH locus. CASE PRESENTATION: We report a patient who, in addition to trisomy 12, carried a rare double-hit translocation characterized by the IGH;BCL3 translocation and an additional clonal IGH;BCL2 translocation involving IGH and another proto-oncogene BCL2, t(14;18)(q32;q21), commonly found in follicular lymphoma. Further single nucleotide polymorphism (SNP) array-based analysis detected a duplication of the 58.8 kb region at 19q13.32 adjacent to the BCL3 translocation junction on chromosome 19q13. Interestingly, the duplicated region contained ERCC2 gene, which encodes a DNA excision repair protein involved in the cancer-prone syndrome, xeroderma pigmentosum. CONCLUSIONS: Taken together our findings indicate the existence of double-translocation driven oncogenic events involving both IGH loci and proto-oncogenes BCL2 and BCL3. Importantly, the IGH;BCL3 translocation was characterized by the duplication of the genomic region adjacent to BCL3, containing a major DNA repair factor, ERCC2.

Burkitt's Lymphoma Presenting as Late-Onset Posttransplant Lymphoproliferative Disorder following Kidney and Pancreas Transplantation: Case Report and Review of the Literature.

Posttransplant lymphoproliferative disorders (PTLD) are a rare, but serious complication following transplantation. Late-onset PTLD are often associated with more monoclonal lesions and consequently have a worse prognosis. There are only isolated case reports of Burkitt's lymphoma presenting as PTLD. We present an extremely rare, aggressive Burkitt's lymphoma years after kidney and pancreas transplantation which was successfully treated with combination chemotherapy along with withdrawal of immunosuppression. The patient remains in complete remission more than 2 years after his diagnosis. We also provide a succinct review of treatment of various PTLD and discuss the role of Epstein-Barr virus infection in the pathogenesis of PTLD.

Cushing's syndrome and nocardiosis associated with a pulmonary carcinoid tumor: report of a case and review of the literature.

Ectopic hormone production is an uncommon complication of neoplastic lung disease. Rarely, patients may present with signs and symptoms of systemic endocrine dysfunction related to a hormone-secreting tumor. Bronchopulmonary carcinoids are the most common neoplasm implicated in ectopic ACTH-dependent Cushing's syndrome. Persistent hypercortisolism, such as that which occurs in Cushing's syndrome, causes immunosuppression and makes patients vulnerable to opportunistic infections. We present a case of a 42-year-old woman diagnosed with ACTH-dependent Cushing's syndrome which was originally thought to stem from a pituitary lesion as interpreted on magnetic resonance imaging. Her symptoms persisted after undergoing hypophysectomy, and further work-up involving a fine needle aspiration of the left lung revealed an ACTH-producing carcinoid tumor. Before treatment could be administered, the patient developed several new suspicious nodules in the left lung that were shown by fine needle aspiration to be infectious in nature. A Gram stain revealed numerous Gram positive branching organisms, and culture of the specimen grew Nocardia asteroides. Her pulmonary infection was treated with antibiotics and she underwent successful ablation of the carcinoid tumor.