Comparison between true and partial hemifacial hypertrophy.

Hemifacial hypertrophy (HFH) is rare and characterized by unilateral enlargement of the head and teeth. Hemifacial hypertrophy is classified as true HFH (THFH) with unilateral enlargement of the viscerocranium, and partial HFH (PHFH) in which not all structures are enlarged. We present a case of THFH and compare and contrast it with a case of PHFH. Hemifacial hypertrophy may cover a wide spectrum of defects or may involve only muscle or bone. Myohyperplasia, reported previously as a separate syndrome, may actually represent a forme fruste of PHFH or THFH. The PHFH patient lead a healthy, normal life without significant psychosocial problems and was reluctant to accept his deformity. Contrastingly, the THFH patient had significant social stigma and compromised health together with major aesthetic morbidity. Treatment planning in THFH is arduous and involves multiple modalities. Therefore, it is imperative to differentiate THFH and PHFH for better understanding and management of the condition.     

Danazol administration after gonadotrophin-releasing hormone analogue reduces rebound of uterine myomas [published erratum appears in Hum Reprod 1998 Mar;13(3):780]

We report the results of administration of danazol after suspension of gonadotrophin-releasing hormone analogue (GnRHa) therapy for uterine myomas. A total of 21 women with uterine myomas was treated with 100 mg danazol for 6 months after GnRHa therapy. Uterine volume and endocrine status were monitored monthly by ultrasound and assay of plasma gonadotrophins, oestradiol and progesterone. The results show a rebound of uterine volume about 30% less than in controls at the end of danazol therapy. Menstrual cyclicity returned after 65 +/- 3 days in 16 subjects and five patients remained amenorrhoeic. Hormone assays confirmed renewed ovarian function in the women whose menstrual periods returned. Bone mineral content was substantially reduced during GnRHa treatment but improved significantly during danazol therapy even in the women who remained amenorrhoeic. These results show the utility of danazol in prolonging the therapeutic effects of GnRHa. The mechanism by which danazol inhibits rebound of uterine volume may be due to its antiprogesterone effects on uterine myomas.      

Contemporary staging and prognosis for primary tracheal malignancies: a population-based analysis.

OBJECTIVE: Determine staging characteristics and survival outcomes for primary malignancies of the trachea. Design Cross-sectional analysis of national cancer database. METHODS: Cases of primary tracheal malignancy were extracted from the Surveillance, Epidemiology, and End Results database for the time period 1988-2000. T-stage, N-stage, and overall stage of presentation were determined. Mean, median, and 5-year survival statistics were computed using Kaplan-Meier survival analysis for each tumor histology and for the overall cohort according to stage. RESULTS: Ninety-two cases with adequate histologic information were identified. Mean age at presentation was 59.3 years with an equal sex distribution. Squamous cell carcinoma was the most common tumor type (41 cases) followed by adenoid cystic carcinoma (19 cases). Forty-nine cases (53%) presented with stage 3 or stage 4 disease. Squamous cell carcinoma exhibited poorer survival (mean survival, 44.0 month, 5-year survival, 34%) than adenoid cystic carcinoma (mean survival, 115 month, 5-year survival, 78%). Five-year unadjusted survival rates according to overall stage were 52.8%, 70.0%, 75.0%, 15.1%, respectively. CONCLUSIONS: Primary tracheal malignancies often present with advanced stage. Patients with squamous cell carcinoma of the trachea have poorer prognoses when compared with adenoid cystic carcinoma and other tumor types. Staging tracheal cancer with a TNM-based system helps predict survival. EBM rating: C.     

Factors affecting survival in maxillary sinus cancer.

PURPOSE: The study goal was to determine survival parameters and clinical factors influencing survival for maxillary sinus malignancies. METHODS: Cases of maxillary sinus malignancy for the time period 1988 to 1998 were extracted from the Surveillance, Epidemiology and End Results database. Cases with distant metastatic disease at presentation were excluded. Clinical information, including tumor histology, grade and stage, and extent of surgery and radiation therapy, were determined. Kaplan-Meier survival and Cox proportional hazards analyses were conducted to determine the influence of these factors on overall survival. RESULTS: The study sample was composed of 650 patients with maxillary sinus cancer (mean age, 64 years; male-to-female ratio, 3:2). The most common histology was squamous cell carcinoma (61.7%), followed by adenoid cystic carcinoma (9.8%). The overall mean (median) survival was 52 months (25 months), and 77.5% and 7.4% of patients presented with advanced (T3/T4) disease or cervical metastasis, respectively. Radiation therapy was administered in 441 patients (67.9%) and significantly improved survival mainly for those with T4 lesions. On multivariate analysis, increasing age, T stage, N stage, and tumor grade independently predicted poorer survival, whereas gender did not. Adenoid cystic carcinoma exhibited a significantly improved overall survival (P <.001). CONCLUSIONS: Survival for patients with maxillary sinus cancer is determined not only by TNM staging but also by tumor histology and grade. TNM staging effectively stratifies patients according to survival. Radiation therapy significantly improves survival for those with T4 lesions.