Oral manifestations of internal malignancy and paraneoplastic syndromes.

Malignant tumors of visceral organs are a fundamental feature of familial cancer and paraneoplastic syndromes. In many instances, the presence of an internal and often occult malignancy may be forewarned by various external manifestations. Several of these findings are preferentially localized to the head and neck region, including the oral cavity proper. This places the dental practitioner in a unique position to detect these "markers" of occult neoplastic involvement. Because these markers may present before an established syndrome or cancer diagnosis, even representing the first expression of disease in some cases, early recognition by a dentist may lead to timely diagnosis and management of these cancer-associated syndromes.

Itch Management: Systemic Agents.

Itch is a global clinical problem and finding effective treatment remains a therapeutic challenge because of the complex pathophysiology of itch. The key component of treating itch should be directed at the underlying etiologies when possible. However, without eradication of the underlying diseases, treatment is often palliative at best. Treatment with systemic therapies can vary according to the etiology of the chronic itch. The aim of this article is to review the major systemic anti-itch agents and give a summary on the possible systemic treatments for different types of itch.

Novel brainstem syndrome associated with prostate carcinoma.

Two patients successfully treated for prostatic cancer developed a progressive neurologic syndrome beginning with loss of voluntary horizontal eye movements followed by severe, persistent muscle spasms of the face, jaw, and pharynx. Both had mild gait unsteadiness, and one exhibited facial and abdominal myoclonus. Extensive diagnostic studies, including MRIs of the brainstem (with and without contrast), were normal. CSF examination showed mild pleocytosis and elevated IgG. Quantitative eye movement recordings documented selective involvement of voluntary horizontal saccades with sparing of horizontal slow eye movements. Neither patient had antineuronal antibodies in the blood. Postmortem examination revealed perivascular chronic inflammatory cells and microglial infiltration of the pons and medulla. One patient also had perivascular infiltrates in both mesial temporal lobes. Neuronal loss was localized to the pontine tegmentum, the medullary sensory nuclei, and the cerebellum. Brainstem motor nuclei were preserved. The clinical and pathologic findings suggest an autoimmune process (probably paraneoplastic) with selective damage to a subpopulation of brainstem neurons critical for horizontal eye movements and recurrent inhibition of bulbar nuclei.

Phosphaturic mesenchymal tumor, mixed connective tissue variant, of the mandible: report of a case and review of the literature.

Tumor-induced osteomalacia (TIO) is a rare paraneoplastic syndrome that results in renal phosphate wasting with hypophosphatemia. In most cases, the underlying cause of TIO is a small mesenchymal neoplasm that is often difficult to detect, resulting in delayed diagnosis. One such neoplasm is the phosphaturic mesenchymal tumor, mixed connective tissue variant (PMTMCT), an unusual entity with unique morphologic and biochemical features. Most of these tumors are found at appendicular sites with only rare cases reported in the jaws. We describe a PMTMCT involving the mandible in a patient with a protracted history of osteomalacia. A review of the current literature is provided with emphasis on the clinical and histologic features, etiopathogenesis, and management of PMTMCT in the setting of TIO.

Pemphigus vulgaris and paraneoplastic pemphigus.

The history, epidemiology, pathophysiology, clinical presentation, diagnostic work-up, histopathology, and treatment of PV and PNP have been presented. These life-threatening, autoimmune, mucocutaneous bullous conditions may be encountered first by oral health providers and, therefore, deserve keen understanding and attention by the oral and maxillofacial surgeon. Great diagnostic and management strides have been made, but morbidity and life quality issues remain a reality for these chronically ill patients.

Budesonide for local therapy of complex oral mucosal immune-mediated inflammatory diseases: case reports.

Local immunosuppressive therapies without systemic effects represent a therapeutic advantage in management of immune/inflammatory oral mucosal conditions. Topical budesonide rinses were prescribed to patients with mucosal disease that was resistant to other intervention without side effects. A case of paraneoplastic pemphigus and a patient with oral graft-versus-host disease that had not responded to standard approaches to management were successfully managed with budesonide rinse application. The cases presented represent mucosal conditions that were successfully managed with topical application of budesonide with reduced risk of systemic effects.

Facial numbness in a man with inguinal and retroperitoneal masses.

BACKGROUND: A 37-year-old Brazilian man was admitted to Massachusetts General Hospital for evaluation of left-sided facial numbness, left-sided ataxia, dizziness, and vertigo. Seven weeks prior to admission, he reported numbness of the left oral cavity and tongue after a dental procedure. Three weeks prior to presentation, he developed left-sided incoordination with dizziness and vertigo. One week later, he noticed difficulty using a box cutter at work and presented to the emergency department for evaluation. A CT scan without contrast revealed no abnormalities and he was discharged home. Three days prior to admission, the patient developed diplopia. Cranial MRI revealed a 1.6 cm irregular enhancing mass in the left middle cerebellar peduncle. Neurologic examination was significant for diminished sensation over the left face to pinprick, left-sided dysmetria, and mild lateral instability of the trunk while walking. INVESTIGATIONS: MRI, lumbar puncture, CT scans of the abdomen and pelvis, needle biopsy of the lymph node, and paraneoplastic antibodies. DIAGNOSIS: Possible paraneoplastic demyelination. MANAGEMENT: Orchiectomy, adjuvant radiation, and corticosteroids.

[Neuropathy of the chin associated with neoplasms. Presentation of 5 cases and a review of the literature]. / Neuropatía mentoniana asociada a neoplasia. Presentación de cinco casos y revisión de la literatura.

Mental neuropathy is a paraneoplastic syndrome characterized by hypoesthesia or anesthesia in the area of mental nerve inervation. Its ethyology is not well known, and in some cases would be secondary to metastasis in the mandible, meningeal carcinomatosis or infiltration of the ganglion of Gasser. We report five new cases; three of them we have studied the presence of circulating antibodies against nervous tissue of ganglion of Gasser, and it was negative in all of them. Likewise we have reviewed the literature.

Paraneoplastic pemphigus in association with Castleman's disease.

BACKGROUND: Paraneoplastic pemphigus (PNP) is an autoimmune mucocutaneous disease associated with lymphoproliferative neoplasms, and frequently with a very rare tumour, Castleman's disease. OBJECTIVES: To analyse the clinical history, immunopathological and histopathological findings in 28 patients with a confirmed diagnosis of PNP and Castleman's disease. METHODS: Sera from all patients were assayed by indirect immunofluorescence (IF) and immunoprecipitation (IP) for plakin autoantibodies, immunoblotting for detection of plectin autoantibodies, and enzyme-linked immunosorbent assay for detection of desmoglein (Dsg)1 and Dsg3 autoantibodies. RESULTS: Severe oral mucositis was observed in all patients, and lichenoid cutaneous lesions were seen in 19 of 28. Twenty cases of Castleman's disease were of the hyaline vascular type, four were of plasmacytoid type and four were of mixed type. Striking findings included pulmonary destruction leading to bronchiolitis obliterans in 26 patients and fatal outcome due to respiratory failure in 22 patients with pulmonary involvement. Histological findings included lichenoid and interface dermatitis with variable intraepithelial acantholysis. Direct IF showed deposition of IgG and C3 in the mouth and skin in 24 of 28 patients. However, indirect IF detected serum IgG autoantibodies in all patients. IP revealed IgG autoantibodies against desmoplakin I, envoplakin and periplakin in all cases, and against desmoplakin II and the 170-kDa antigen in 19 patients. Dsg3 and Dsg1 autoantibodies were present in 22 and 11 patients, respectively, and plectin autoantibodies in 23 patients. CONCLUSIONS: PNP in association with Castleman's disease presents with severe oral mucositis and cutaneous lichenoid lesions. Serum autoantibodies against plakin proteins are the most diagnostic markers. Pulmonary injury with respiratory failure is the cause of death in most cases.