Life-threatening hypoglycemia resulting from a nonislet cell tumor.

Nonislet cell tumor-induced hypoglycemia (NICTH), also known as Doege-Potter syndrome, is a rare paraneoplastic syndrome seen in association with various nonpancreatic tumors, benign and malignant, and comprising mesenchymal, vascular, or epithelial cell types. We report a case of recurrent life-threatening hypoglycemia from a large pelvic solitary fibrous tumor.

Ameloblastic carcinoma with features of ghost cell odontogenic carcinoma in a patient with suspected Gardner syndrome.

Ameloblastic carcinoma and ghost cell odontogenic carcinoma are rare malignancies arising in odontogenic epithelium within the jaws. Gardner syndrome is a multifaceted autosomal dominant condition, which results in multiple dentofacial anomalies along with premalignant colon polyp formation and tumor formation in the skin and other organs. We report a case of ameloblastic carcinoma with features of ghost cell odontogenic carcinoma and extensive clear cell change and melanin pigmentation in a patient with clinical features of Gardner syndrome.

Glycoengineering in cancer therapeutics: a review with fucose-depleted trastuzumab as the model.

Experimentally modified trastuzumab antibodies show increased cytotoxic potency when used with human effector cells against HER2-overexpressing human breast cancer cells in vitro and ex vivo. Furthermore, the superior efficacy of 'glycoengineered' trastuzumab has been confirmed in vivo utilizing a preclinical xenograft model of human HER2-amplified, trastuzumab-resistant human breast cancer. The increased cytotoxic potency coupled with other improvements are achieved by a seemingly modest change in trastuzumab's structure, that is, depletion of two α-L-fucose residues from trastuzumab's heavy chains.

The emerging importance of α-L-fucose in human breast cancer: a review.

Breast cancer cells incorporate the simple sugar alpha-L-fucose (fucose) into glycoproteins and glycolipids which, in turn, are expressed as part of the malignant phenotype. We have noted that fucose is not simply a bystander molecule, but, in fact, contributes to many of the fundamental oncologic properties of breast cancer cells. Here, we summarize the evidence from us and others that fucose is necessary for key functions of neoplastic progression including hematogenous metastasis, tumor invasion through extracellular matrices including basement membranes and up-regulation of the Notch signaling system, with implications for epithelial-to-mesenchymal transition and activation of breast cancer stem cells.

Systemic CD5+ MALT lymphoma: presentation with Waldenstrom syndrome.

We report a case of extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) in a 75-year-old woman with a neuropathy related to high levels of serum immunoglobulin M and a history of rheumatoid arthritis and polymyositis. The patient developed a mass in the right submandibular salivary gland, and this mass demonstrated histopathologic features that are typical of MALT lymphoma, including infiltrates of small monocytoid B cells in the epithelium (forming "lymphoepithelial lesions"), a reactive background of florid germinal center hyperplasia, and follicular colonization by the monocytoid B cells. Many plasma cells in the background expressed cytoplasmic immunoglobulin M lambda, matching the serum spike.

An unusual presentation of Teflon granuloma: case report and discussion.

For more than 25 years, Teflon was the most commonly used material for injection laryngoplasty. However, the incidence of Teflon granuloma and the consequent deterioration of glottic function ultimately led to the development of other injectable materials, and as a result, Teflon granulomas are no longer frequently encountered. We present a case of Teflon granuloma that was unusual in that (1) a long period of time had elapsed between the injection and the granuloma formation and (2) there was no change in the patient's glottic function.

Cell surface associated alpha-L-fucose moieties modulate human breast cancer neoplastic progression.

Glycosylation drives critical processes important for mammalian cell-cell and cell-matrix interactions. Alpha-L-fucose (alpha-L-f) is a key monosaccharide component of oligosaccharides that has been found to be overexpressed during tumor progression. Modification of cell surface fucosylation, we hypothesized, alters tumor cell phenotype and function at the end of the neoplastic progression cascade including tumor invasion.

Alterations in human breast cancer adhesion-motility in response to changes in cell surface glycoproteins displaying alpha-L-fucose moieties.

Glycosylation of proteins plays multiple roles in cell-cell and cell-matrix interactions. Fucose is a monosaccharide associated with glycosylation events and is known to be over-expressed in many malignant tumors. By using alpha-L-fucosidase (alpha-L-fase), a glycosidase that specifically removes alpha-L-fucose (alpha-L-f), we have examined the potential effects of defucosylation on tumor functions, focusing on tumor progression in the context of the interaction of tumor cells with the extracellular microenvironment.

Expression of HGAL in primary cutaneous large B-cell lymphomas: evidence for germinal center derivation of primary cutaneous follicular lymphoma.

The classification of primary cutaneous large B-cell lymphoma (PCLBCL) is based on standard morphology, immunohistochemistry, and clinical presentation. There are two major subtypes in the current WHO-EORTC classification: follicle center lymphoma and diffuse large B-cell lymphoma, leg-type (DLBCL-LT). The goals of this study were to examine a series of DLBCLs to determine (1) whether the immunohistochemical paradigm of germinal center B-cell and non-germinal center B-cell types of systemic DLBCL could be applied to PCLBCL; (2) whether application of the newly described germinal center B-cell marker, human germinal center-associated lymphoma (HGAL) also discriminates between these types as a further support for germinal center B-cell origin for primary cutaneous center lymphoma; and (3) whether any of these biologic markers were of prognostic significance.

An intriguing case: malignant mesothelioma presenting as inguinal lymph node metastases.

A 56-year-old woman presented with a right inguinal mass. Biopsy revealed multiple lymph nodes involved with papillary and gland-like structures extending into the surrounding fibroadipose tissue. There was no history of carcinoma or other malignancy.

A practical approach to the diagnosis of Hodgkin lymphoma.

The diagnosis of Hodgkin lymphoma has become more difficult in recent years, paradoxically because of great strides made in the understanding of the molecular mechanisms driving the lymphomas. Extensive panels of monoclonal antibodies, coupled with flow cytometric analyses and molecular diagnostic studies, have served to define more lymphoma subtypes that cannot be identified based on morphologic evidence alone. This overview is intended to provide useful criteria for the recognition of Hodgkin lymphoma and to recommend tools that will aid in separating Hodgkin lymphoma from a series of common benign and malignant look-alikes.

Multiple Langerhans cell nodules in an incidental thymectomy.

The isolated appearance of Langerhans cell histiocytosis in the thymus is quite rare. We report the finding of multiple small Langerhans cell nodules in the thymus of an 11-month-old infant who had undergone thymectomy in the course of surgical correction of tetralogy of Fallot. The subtle appearance of some of these nodules and aggregates was unlike the pattern of massive thymic infiltration seen in 7 previously reported cases of thymic Langerhans cell histiocytosis in childhood.

Composite mycosis fungoides and B-cell chronic lymphocytic leukemia.

Concordant or composite mycosis fungoides and B-cell chronic lymphocytic leukemia (B-CLL) is exceedingly rare, with only 10 cases previously described to our knowledge. We report a case of a 64-year-old woman who developed generalized erythroderma 5 years after the diagnosis of early stage B-CLL. Over the next 6 years of her clinical course multiple sequential samples of skin, peripheral blood, and one enlarged lymph node were studied in detail by flow cytometry, immunohistochemistry, molecular diagnostics, and electron microscopy.

Concomitant sclerosing mesenteritis and bile duct fibrosis simulating Klatskin's tumor.

Sclerosing mesenteritis is an uncommon benign condition that should be included in the differential diagnosis of abdominal masses. We present the first reported case of this condition in association with idiopathic bile duct fibrosis simulating Klatskin's tumor. A review of the literature regarding both clinical entities is presented.

Cell surface fucose ablation as a therapeutic strategy for malignant neoplasms.

The sugar alpha-L-fucose is overexpressed in many human malignancies, especially on specific glycoproteins, glycolipids, certain mucins, and putative cell adhesion ligands found on cancer cell surfaces. Many of these molecules are known or suspected mediators of cell-cell adhesion, cell signaling, motility, or invasion. As knowledge of fucose metabolism evolves and specific mechanisms of its distribution and incorporation are more exactly documented, modulation of fucose expression in cancer is becoming increasingly more feasible.

MALT lymphoma presenting as a cystic salivary gland mass.

Background: Mucosa associated lymphoid tissue (MALT) lymphoma has been noted to involve the salivary glands in chronic inflammatory conditions such as Sjogren's syndrome and in HIV infection.

Methods And Results: The authors encountered a patient with bilateral cystic changes in the parotid glands which proved to be due to MALT lymphoma. The clinical course, histopathology, and treatment options of MALT lymphoma in the salivary gland are discussed in detail.

A prospective trial of telepathology for intraoperative consultation (frozen sections).

Telepathology is a maturing technology that, for a variety of reasons, has not been widely deployed. In addition, clinical validation is relatively modest compared with accepted telemedicine applications such as teleradiology. A prototype telepathology system (Tele-Path(sm)) featuring high-resolution images selected from a remote microscope site has been developed at the University of Alabama at Birmingham (UAB).

Hodgkin's and non-Hodgkin's lymphoma: spectrum of morphologic and immunophenotypic overlap.

Immunoperoxidase markers are useful and often essential in distinguishing among lymphocyte-predominant Hodgkin's lymphoma, T-cell-rich B-cell lymphoma, and lymphocyte-rich classic Hodgkin's lymphoma. However, it is becoming increasingly clear that these "entities" are closely related clonal B-lineage neoplasms that may intertransform and/or coexist. We hypothesized that, just as there are cases with morphologic overlap, there would also be immunophenotypic overlap that would be found when a series of such cases is studied in detail.

Transforming growth factor alpha (TGF-alpha) expression in dysplastic oral leukoplakia: modulation by 13-cis retinoic acid.

Background: Surrogate endpoint biomarkers (SEBs) are detectable molecular, cellular, and tissue changes that take place during tumorigenesis and can be modulated by a chemoprevention agent.

Method: To identify candidate SEBs for invasive squamous cell carcinoma of the upper aerodigestive tract (SCC), we have studied the expression of transforming growth factor-alpha (TGF-alpha) and epidermal growth factor receptor (EGFr) in sequential biopsy specimens of dysplastic oral leukoplakia and adjacent normal-appearing mucosa. Biopsies were taken from patients before, during, and after treatment with 13-cis retinoic acid, a vitamin A derivative.

Composite B-cell and T-cell lymphoma arising 24 years after nodular lymphocyte predominant Hodgkin's disease.

Twenty-four years after apparently successful treatment for nodular lymphocyte predominant Hodgkin's disease (nLPHD), a 41-year old male developed "B" symptoms and extensive adenopathy. A right axillary lymph node biopsy showed two distinct regions including (1) histiocyte-rich B-cell lymphoma and (2) diffuse small T-cell lymphoma. A clonal rearrangement of the gene for the T-cell receptor beta chain confirmed the presence of a T-cell neoplasm, and this was further confirmed by selective polymerase chain reaction (PCR) on this morphologic zone.

Alpha-L-fucose: a potentially critical molecule in pathologic processes including neoplasia.

Alpha-L-fucose is a 6-carbon deoxyhexose that is commonly incorporated into human glycoproteins and glycolipids. It is found at the terminal or preterminal positions of many cell-surface oligosaccharide ligands that mediate cell-recognition and adhesion-signaling pathways. These include such normal events as early embryologic development and blood group recognition and pathologic processes including inflammation, infectious disease recognition, and neoplastic progression.

An initial trial of a prototype telepathology system featuring static imaging with discrete control of the remote microscope.

Routine diagnosis of pathology images transmitted over telecommunications lines remains an elusive goal. Part of the resistance stems from the difficulty of enabling image selection by the remote pathologist. To address this problem, a telepathology microscope system (TelePath, TeleMedicine Solutions, Birmingham, Ala) that has features associated with static and dynamic imaging systems was constructed.