Tumor pardo del paladar, presentación atípica de hiperparatiroidismo primario en adolescente: buena evolución y normalización de la masa ósea tras paratiroidectomía / Brown tumor of the palate, atypical presentation of primary hyperparathyroidism in an adolescent: good evolution and bone mass normalization after parathyroidectomy

El hiperparatiroidismo primario (HPTP) es poco frecuente en niños y adolescentes. Hay escasos datos para el manejo de estos pacientes en pediatría. Las glándulas paratiroideas son glándulas endocrinas que secretan hormona paratiroidea (PTH) y regulan el metabolismo del calcio y del fósforo. La sobreexpresión de PTH se llama hiperparatiroidismo, que se clasifica en primario, secundario y terciario. En los adolescentes, 80 a 92% de los hiperparatiroidismos primarios se deben a adenoma paratiroideo. Presentamos el caso clínico de una adolescente con una primera manifestación atípica de HPTP, la presencia de un tumor pardo del paladar, presentación rara de adenoma paratiroideo, acompañado de hipercalcemia, marcada elevación de PTH y varias lesiones óseas. (AU)

Primary hyperparathyroidism (PHPT) in children and adolescents is uncommon. Guidelines for management in pediatric patients are limited. Parathyroid glands are endocrine glands that secrete parathyroid hormone (PTH) and regulate calciumphosphate metabolism. The overexpression of PTH is called hyperparathyroidism, and is classified as primary, secondary, and tertiary. In adolescents, 80 to 92% of PHPT cases are due to a parathyroid adenoma. We present here a case report of an adolescent with a brown tumor of the palate as the first manifestation of the disease, atypical and rare presentation of parathyroid adenoma in an adolescent. She had hypercalcemia, marked elevation of PTH and bone lesions. (AU)

The localization and risk factors of squamous cell carcinoma in the oral cavity: A retrospective study of 1501 cases.

Head and neck cancer is the tenth leading cause of cancer mortality. Ninety percent of tumours in the oral cavity are squamous cell carcinomas. Information about the exact localisation of OSCC is missing in the literature. In the present study, we retrospectively analysed a total of 1501 OSCC patients, who were treated between 1975 and 2009. The purpose of this study was to examine the localisation of OSCC tumours and to analyse the influence of various parameters on tumour localisation. 71.5% of these patients were male and 28.5% were female. The mean age was 60 years. The most common sites of OSCC occurrence were the floor of the mouth and the anterior base of the mouth. The hard palate was the most affected anatomical area of the maxilla. Descriptive statistical analysis, chi-square testing and a multivariate analysis using a multinomial logistical model showed a significant correlation of younger age and female gender with tumour occurrence in the maxilla and the tongue. We provide a very detailed anatomical mapping of OSCC.

Intraoral lesion · history of cirrhosis and smoking · Dx?

A 56-year-old white man presented at our dental clinic for routine care. The intraoral examination revealed an asymptomatic red lesion with white vesicle-like areas on the right side of the soft palate. The extraoral examination was normal, and regional lymph nodes were nonpalpable. The patient's medical history included liver cirrhosis and pancreatitis. He also had a 30-year history of alcohol misuse (1-5 drinks per day) and a 30-pack-year smoking history. (The patient had stopped drinking at the time of presentation, and had quit smoking 2 years earlier.) We instructed him to gargle with warm salt water at home and return in 2 weeks. At follow-up, the lesion was unresolved, so a biopsy was performed.

Early Arising Sarcoma After Adjuvant Radiotherapy for Oral Squamous Cell Carcinoma.

Radiation-induced sarcoma of the head and neck (RISHN) is a rare and long-term complication of radiation therapy (RT). This report describes a case of RISHN characterized by early and insidious onset. An 80-year-old man was surgically treated for advanced oral squamous cell carcinoma of the left retromolar trigone (pT4aN0). Sixteen months after completion of adjuvant RT, an exophytic sessile lesion arose in the left border of the soft palate. Histologic assessment showed a malignant neoplasm with spindle-shaped cells and areas of bone matrix without perivascular or perineural invasion; such features in addition to immunohistochemical assessment (negative for pan-cytokeratin; positive for vimentin; negative for epithelial membrane antigen; negative for p63; Ki-67, 30%) are consistent with poorly differentiated sarcoma (cT1aN0M0). Fifteen months after a wide surgical resection, the patient was free of disease. RISHN is usually an aggressive neoplasm with insidious onset. Nevertheless, early diagnosis followed by complete surgical excision could make the prognosis comparable to that of spontaneous sarcoma.

Multiple unrelated malignancies following renal transplantation: an evaluation of four cases.

The risk of de novo malignancy is significantly higher in patients who have undergone organ transplantation than in the general population. Long-term immunosuppressive treatment, in addition to age, genetic predisposition and infectious agents, plays a major role in the development of malignancy. Although skin and hemopoietic system cancers are common, atypical presentations of malignancies may occasionally be seen during long-term follow-up in patients with functioning allografts. In this report, four cases, each with more than one different primary malignancy (one patient with three malignancies and three patients with two malignancies), are presented.

Evidence for a possible anatomical subsite-mediated effect of tobacco in oral potentially malignant disorders and carcinoma.

PURPOSE: To test the hypothesis that cigarette smokers develop oral potentially malignant disorders or carcinomas in preferential anatomical subsites. METHODS: The association of smoking habit with the presence of oral lesions in specific anatomical subsites was assessed in 123 patients using the odds ratio analysis. RESULTS: When compared to all the other subsites, the relative frequency of smokers with lesions was higher in the buccal mucosa and in the floor of the mouth (FOM) (P=0.002 and P=0.005), while it was lower in the tongue (P<0.0005). Smokers were about 7 years younger than non-smokers (P=0.008). CONCLUSIONS: The association of smoking and age suggests that smoking may contribute to generate a field of injury that leads to lesions in shorter periods than other causes. The stronger relationship of smoking with lesions in the buccal mucosa and FOM than in the tongue suggests that tissue characteristics mediate the effects of tobacco.

Squamous cell carcinoma on the palate in a patient with systemic lupus erythematosus: case report and review of literature.

Squamous cell carcinoma is a rare consequence of lupus erythematosus and it is generally associated with skin lesions rather than with oral mucosa. This paper reports a patient diagnosed with systemic lupus erythematosus who developed a squamous cell carcinoma on the palate as an outcome of a persistent ulcer, a frequent lesion in patients with this disease and, in fact, clinical criterion for its diagnosis. A 38-year-old female patient diagnosed with systemic lupus erythematosus 12 years previously, who attended the dental school for routine dental control. The patient was being treated with prednisone 10 mg per day and cyclophosphamide 750 mg per month until 10 months prior to her diagnostic biopsy. She had, however, been previously treated with chloroquine. Oral lesions started 6 months before consultation as symptomatic multiple ulcers on the palate. After topical treatment with steroids for 1 month, the lesions regressed except for the central lesion, from which an incisional biopsy was taken and a well differentiated squamous cell carcinoma was diagnosed. The oncological phase consisted of partial palatectomy. To date, 3 years after surgery, the patient is free from malignant lesions. Lupus erythematosus is considered a potentially malignant disorder, although the cause for neoplasic transformation in these patients is still not clear, but cyclophosphamide consumption may be implicated; the case emphasizes the importance of periodical oral evaluation of such patients. Repeated biopsies should be performed if there is failure to respond to conventional therapy.

Human immunodeficiency virus-associated Kaposi sarcoma as an immune reconstitution inflammatory syndrome: a literature review and case report.

BACKGROUND: Kaposi sarcoma (KS) is the most common human immunodeficiency virus (HIV)-associated neoplasm (HIV-KS). Highly active antiretroviral therapy (HAART) results in a decrease in the incidence and prevalence of HIV-KS as well as in clinical improvement. However, in a subset of subjects who are HIV seropositive, KS may recrudesce early following the introduction of HAART as an immune reconstitution inflammatory syndrome (IRIS). METHODS: The management of a patient who is HIV seropositive with rapid clinical worsening of oral KS lesions shortly after the initiation of HAART was documented. Repeated serologic testing for CD4(+) T-cell count and microscopic examination of two biopsy specimens of the oral lesion, one taken before and the other taken after cytotoxic chemotherapy, followed by surgical excision was the treatment modality used. RESULTS: Microscopic examination of the incisional biopsy specimen taken from the oral lesion at the time of the initial consultation confirmed the clinical diagnosis of KS. The sequential serological tests showed a progressive increase in CD4(+) T-cell counts that paralleled the rapid clinical worsening of the KS disease. This was consistent with the diagnosis of IRIS-associated HIV-KS. Subsequent cytotoxic chemotherapy brought about resolution of the IRIS and regression of the HIV-KS lesions. Microscopic examination of a biopsy specimen obtained after cytotoxic chemotherapy did not show any of the original KS. The residual palatal exophytic mass was excised. CONCLUSIONS: IRIS-associated HIV-KS is not a disease, but rather a temporary paradoxical immunoinflammatory reaction brought about by improvement in immune status following HAART. IRIS-associated HIV-KS can be controlled effectively by limited systemic cytotoxic chemotherapy in the setting of HAART.

Cavernous hemangioma of the palate. A review of etiology, pathogenesis and treatment options.

Cavernous hemangiomas of the palate are a rare but not infrequent sighting in the oral and maxillofacial region. The etiology and pathogenesis of these lesions are not definitively understood and are currently being explored. Treatment options usually include, but are not limited to, surgical excision of the lesion. Alternative management paradigms should be considered when dealing with this type of lesion. Precaution should be taken before any surgical intervention.

Adult T-cell leukemia/lymphoma with predominant bone involvement, initially diagnosed by its oral manifestation: a case report.

Adult T-cell leukemia/lymphoma (ATL/L) is a rare malignant neoplasm linked to human T-cell lymphotropic virus type 1 (HTLV-1). This virus has been identified in Japan, the Caribbean, and, more recently, Brazil. We report a case of ATL/L (lymphoma-type) affecting a 30-year-old Brazilian woman. She presented a painful and ulcerated lesion on her hard palate mucosa. Conventional radiographs and computed tomography revealed the involvement of several bones plus the lung and axillary lymph nodes. Histopathological and immunohistochemical analyses of oral biopsy confirmed a T-cell non-Hodgkin's lymphoma. Final diagnosis of ATL/L was made based on HTLV-1 positivity. She underwent multiple cycles of chemotherapy, which produced some improvement, but she died as a consequence of pulmonary and hepatic complications 4 months after the initial diagnosis. Besides the process of diagnosing and typing a malignant lymphoma, this article outlines the value of computed tomography and the necessity of performing HTVL-1 investigation in patients with a diagnosis of lymphoma of T-cell lineage.

No pharmacokinetic drug-drug interaction between nevirapine and paclitaxel.

We have investigated the pharmacokinetics of nevirapine and paclitaxel in a patient who used both drugs concomitantly, as there are strong theoretical indications for a potential pharmacokinetic drug-drug interaction. Plasma concentrations of nevirapine (dose: 200 mg twice daily orally) and paclitaxel (dose: 100 mg/m(2) 3-h i.v. infusion) were determined in a HIV-1-infected patient with Kaposi's sarcoma. Since both drugs are metabolized via the same cytochrome P450 isoenzymes, investigation of a drug-drug interaction was considered important. We found that the plasma concentrations of nevirapine given together with paclitaxel were similar to those given without paclitaxel. The exposures to paclitaxel (AUC(0-infinity) = 3787 h.ng/ml) and its hydroxy metabolites when co-administered with nevirapine were comparable to the mean exposure to paclitaxel and its metabolites from eight historical controls (AUC(0-infinity) = 3614 h.ng/ml) treated with the same dose. No pharmacokinetic drug-drug interaction between nevirapine and paclitaxel could be demonstrated in our HIV-1-infected patient.

Common benign oral soft tissue masses.

This article reviews some of the more common benign oral soft tissue masses with emphasis on their etiology, epidemiology, clinical presentation, histopathology, and treatment. These lesions include traumatic fibroma, mucocele, warts/papilloma, pyogenic granuloma, peripheral giant cell granuloma, generalized gingival hyperplasia, gingival fibromatosis, lateral periodontal cyst, lipoma, and denture-induced hyperplasia.

[Triple secondary malignancy of gingiva, palate and esophagus after an allogeneic bone marrow transplantation for cutaneous T-cell lymphoma].

A 31-year-old man was diagnosed as having cutaneous T-cell lymphoma in January 1994. He received an allogeneic bone marrow transplantation (BMT) from an HLA-matched sibling donor in May 1995, because of refractoriness to chemotherapy. The patient had been treated with immunosuppressants including prednisolone and cyclosporin A for chronic graft-versus-host disease (GVHD) of the extensive type following acute GVHD. Five years after the BMT, he developed moderately differentiated squamous cell carcinoma (SCC) on the mandibular gingival mucosa and underwent surgical resection. Furthermore, 6 years after the BMT well differentiated SCC developed on his palate and was resected. Concurrently, he was diagnosed as having esophageal cancer (poorly differentiated SCC) and underwent a subtotal esophagotomy. One year later he had a recurrence of the esophageal cancer with dysphagia and was treated with radiation and chemotherapy. He remains free of triple cancer and lymphoma. It is suggested that total body irradiation, immunosuppressants, and chronic GVHD are associated with a risk of secondary malignancies following allogeneic BMT. These factors might have contributed to the onset of triple cancer in our patient.

Primary hyperparathyroidism presenting as a palatal brown tumor.

Only 2% of all primary hyperparathyroidism cases occur in patients below the age of 30 years. Owing to the improved methods of blood analysis in the last 2 decades, most of the recent cases of primary hyperparathyroidism are diagnosed early and asymptomatically. This makes advanced disease with bone lesions extremely rare these days. A case of a 25-year old female patient with previously undiagnosed primary hyperparathyroidism who presented first with palatal swelling is reported. Further investigations revealed parathyroid adenoma, nephrocalcinosis, and extensive bone lesions involving the maxillary sinus and palate as well as the pelvic bone. The evaluation of the hyperparathyroid patient is reviewed in this report. The methods of treatment of the bony lesions of primary hyperparathyroidism are also discussed.

Oral Kaposi's sarcoma in a renal transplant patient: case report and literature review.

Malignancies, including oral Kaposi's sarcoma, may develop in transplant patients as a result of immunosuppressive therapy. Both the prevalence and the incidence of these malignancies vary. This article describes a renal transplant patient who was receiving immunosuppressive therapy and presented with oral Kaposi's sarcoma. The lesion was excised and did not recur. However, the patient died as a result of viral pneumonitis, secondary to her renal problems. The article also includes a review of the literature, with particular emphasis on oral presentation of immunosuppression-related malignancies.

Sigmund Freud: smoking habit, oral cancer and euthanasia.

BACKGROUND: Sigmund Freud, the father of modern psychoanalysis had a well-known love of the cigar. The natural progression of this vice was the development of oral cancer for which he underwent a lengthy ordeal. An account is given in this article of Sigmund Freud's illness and care following the diagnosis of his oral cancer. The role of euthanasia and physician assisted suicide is also discussed. METHODS: A review of relevant literature on Sigmund Freud's illness, risk factors for oral cancer and euthanasia was undertaken. RESULTS: Sigmund Freud was a heavy smoker with a 20-cigar/day habit. In 1923, a diagnosis of squamous cell carcinoma of the palate was made, for which he underwent a lengthy ordeal which span a total of 16 years. During this period, he bluntly refused to quit smoking. Freud consulted many specialists (otolaryngologists, oral and maxillofacial surgeons, prosthodontists and general surgeons), during the course of his ordeal with oral cancer. He underwent 34 surgical procedures before his eventual death in 1939 through euthanasia. CONCLUSION: Continued indulgence in smoking and procrastination on the part of Freud, as well as mediocrity, negligence and incompetence on the part of the first surgeon that operated on Freud, could partly be responsible for his lengthy ordeal.

Focal dermal hypoplasia: management of complex dental features.

A 17 year-old female who presented for treatment of grossly carious lower first molar teeth had multiple features of Focal Dermal Hypoplasia (FDH). These included enamel pitting and hypoplasia, anomalies of shape, size and positioning of teeth, as well as soft tissue papillomas and telangiectasis of tongue. A case report and a synopsis of the syndrome (FDH) are presented, the oral aspects of the condition are reviewed and the management of the complex dental anomalies is discussed.

[Primary hyperparathyroidism presenting as a brown tumor of the maxilla and hard palate. Apropos of a case]. / Hyperparathyroïdie primaire révélée par des tumeurs brunes des maxillaires et du palais osseux. A propos d'un cas.

Significant controversy persists in the classification of giant cell lesions of the maxillofacial skeleton. Multifocal giant cell lesions are strongly suggestive of hyperparathyroidism brown tumors. Maxillary localization is extremely rare. We report a case of primary hyperparathyroidism manifesting in the form of giant cell tumors of the maxilla, the hard palate and long bones. Biochemistry confirmed the diagnosis. Computed tomography localized the parathyroid tumor at the cervicothoracic junction in the retrosternal area. We discuss the clinical features of skeletal involvement in primary hyperparathyroidism and stress the need for ionized serum calcium assay in case of giant cell tumors of the cranial bones.