[Nasal cavity mucosal melanoma with skin extension mimicking a malignant lentigo with mucosal extension]. / Mélanome muqueux des fosses nasales avec extension cutanée mimant un lentigo malin cutané d'extension muqueuse.

Mucosal melanoma is a rare malignant disease developed from melanocyte. We report the case of a patient with nasal cavity mucosal melanoma with a primary clinical and histological diagnosis of malignant lentigo with mucosal spreading. The presence of a c-Kit mutation, in a second lecture and the evolving nature of the lesion, reorientated the diagnosis of malignant lentigo to mucosal melanoma with skin extension. Extensive surgical resection and foramen free flap with costal graft reconstruction may have a local control of the disease. Yet, after one year, a regional evolution involving a parapharyngeal node was treated by stereotaxic radiotherapy. After 5 years, the patient was considered in clinical and radiological remission. Malignant lentigo with mucosal extension is a very rare situation, this diagnoses must be evoqued after setting mucosal melanoma diagnosis.

Early corrected serum calcium value can predict definitive calcium serum level after total thyroidectomy in asymptomatic patients.

INTRODUCTION: Hypocalcemia is the most common complication of thyroidectomy, requiring supplementation as well as prolonged hospitalization. Our study's objective was to determine a corrected calcium (CCa) level on day 1 after thyroidectomy predictive of no calcium and vitamin supplementation. MATERIALS AND METHODS: A single-center prospective study conducted between January 2012 and July 2015 in 396 patients, consisting of 331 cases of total thyroidectomy, with seven completion surgeries. The data collected were age, sex, type of thyroid surgery, etiology, anatomical pathological analysis, and the need for calcium and vitamin supplementation therapy as well as its duration. CCa levels were analyzed 20 and 30 h after surgery then on days 2 and 3. To determine a cut-off value for CCa, a ROC curve analysis was performed. The population was described in terms of numbers and associated percentages for categorical variables, and mean. RESULTS: Mean CCa on 20 h after surgery was 2.09 mmol/L (p < 0.001) and 30 h was 2.06 mmol/L p = 0.02. CCa of less than 2.13 mmol/L was predictive of calcium and vitamin supplementation with 56% sensitivity and 97% specificity. On the evening of day 1, the cut-off value for CCa was 2.06 mmol/L with 67% sensitivity and 65% specificity. CONCLUSION: This prospective study confirms that CCa on the first morning after surgery is reliable when it is more than 2.13 mmol/L. In total, analyzing CCa on day 1 after total thyroidectomy allows the discharge of 70% of patients on the first day after surgery, with no risk of hypocalcemia.

Prognostic value of two tumour staging classifications in patients with sinonasal mucosal melanoma.

INTRODUCTION: Sinonasal mucosal melanoma is a rare disease associated with a very poor prognosis. The purpose of this study was to assess the prognostic value of the 2 staging systems published in the literature for these tumours: the American Joint Committee on Cancer (AJCC) Cancer Staging Manual for mucosal melanoma of the head and neck published in 2009 (7th edition) and the AJCC Cancer Staging Manual for cancers of the nasal cavity and paranasal sinuses published in 2002 (6th edition) and the prognostic value of tumour site, either limited to the nasal cavities or with paranasal sinus invasion. METHODS: A retrospective study was conducted on 18 patients treated between August 1998 and June 2014. Each lesion was staged according to the AJCC Cancer Staging Manual 2002 and 2009 and the following data were collected: age, sex, tumour site, initial symptoms, treatment modalities, follow-up, recurrences and overall survival. Patient survival, from the date of discovery of the melanoma until death, was analysed by Kaplan-Meier survival curves and between-group comparison of survival was performed with a log rank test. RESULTS: The mean age at diagnosis was 72 years (range: 54-94) and the cohort comprised 11 women and 7 men. The median overall survival was 80 months, the 1-year overall survival was 82.6% and the 5-year overall survival was 54.5%. The AJCC 2002 staging system presented a statistically significant prognostic value (P=0.0476), while no statistically significant prognostic value was observed for the AJCC 2009 staging system (P=0.108). Paranasal sinus invasion was significantly associated with a poor prognosis (P=0.0039). CONCLUSION: This study demonstrates the superiority of the non-specific AJCC 2002 Cancer Staging Manual. Medical and surgical management must take paranasal sinus invasion into account, as it constitutes a major prognostic factor.

Mandibulo-facial dysostosis: comparison study of a neonate with mandibulo-facial dysostosis and a normal neonate.

Mandibulo-facial dysostosis (MFD) is a malformative syndrome with autosomal dominant transmission and variable expressivity that mainly affects derivatives of the first and second branchial arches. The subsurface anatomy of this condition is still partly unexplored since there have been only four reported dissections of MFD. A detailed dissection of the head and neck of a neonate with MFD is described and compared with a normal neonate. Theories of the pathogenesis are discussed on the basis of these observations.

Mucosal melanoma of the nasal cavity and paranasal sinuses.

Mucosal melanoma of the nasal cavity and paranasal sinuses is a rare disease, but its incidence appears to be increasing. The mean age at diagnosis is between 65 and 70 years. Unilateral nasal obstruction and epistaxis are the most common presenting complaints. Melanoma arises in the septum or lateral wall of the nasal cavity in the great majority of cases. The histological diagnosis is based on specific immunohistochemical labelling and is usually established at an advanced stage of disease: stage T3 or T4 tumours according to the 7th edition of the American Joint Committee on Cancer (AJCC) classification of tumours. First-line treatment consists of surgery. The place of intranasal endoscopic surgery remains controversial due to the difficulty of controlling surgical margins and should be reserved for experienced teams. Adjuvant radiotherapy is usually performed due to its efficacy on local and regional disease control. Five-year overall survival of mucosal melanoma of the nasal cavity and paranasal sinuses in the most recent series does not exceed 40%. Local recurrence is observed in about 50% of cases and metastatic disease is common. The quality of initial tumour resection with negative surgical margins is the most important prognostic factor for tumours confined to the nasal cavity. Hopes for improvement of survival are based on early diagnosis, progress in radiotherapy techniques and cell and gene therapy that are currently under evaluation.

The left recurrent laryngeal nerve at birth: anatomy and surgical applications.

The aim of this study was to define the origin, course, caliber and relations of the left recurrent laryngeal nerve (N Laryngeus recurrens) in order to localize the nerve during surgery for esophageal atresia. Eighteen anatomic specimens were dissected and 12 surgical cases were analysed. Left recurrent laryngeal nerve palsy after this particular form of neonatal surgery is a very real risk. It seemed useful to describe an anatomic technique of identifying the nerve in the mediastinum during such surgery, in order to prevent this complication.

Diameter of the superior vena cava in the newborn: clinical applications in parenteral nutrition.

The authors measured the superior vena cava in the newborn by radiologic, anatomic and histologic methods and analysed the correlations between cross-sectional area and the biometric data. This method makes it possible to select the size of catheter for parenteral nutrition via the superior vena cava in relation to body-weight.

Histological assessment of gestational age in human embryos and fetuses.

A light microscopic study performed on 760 human fetuses allows us to define several stages in their visceral development and to relate these to gestational age. The histological examination of most viscera, such as the central nervous system, is useless when the tissue preservation is poor. Nevertheless, three organs may still be studied in macerated fetuses: the lungs (where different glandular, canalar, and alveolar stages of development are evident), the kidneys (where the respective numbers of rows of primitive glomeruli and of generations of mature glomeruli vary after week 22), and the adrenal glands (where neuroblastic nests are normally found between weeks 7 and 26 of gestation). It is important to compare the degree of histological maturation with the clinical, macroscopic, and radiologic data in order to define the profile of maturation proper to each human fetus.

The anatomy of intersexuality.

A description of anatomical lesions in the intersexual states has been made based on clinical and anatomical observations. This study provides the anatomical basis which is necessary before surgical genital reconstruction can be carried out.

Temporomandibular region in the Franceschetti's Syndrome. Anatomical study.

Franceschetti's syndrome is a rare, non-fatal, hereditary malformation, usually bilateral, which symmetrically affects orbits, mandible and ear. The authors propose an anatomical description of the temporomandibular region after the dissection of a newborn baby suffering from Franceschetti's Syndrome, dead soon after the birth. A discussion on the different etiopathogenical theories is made. The authors conclude that an alteration of the development of nerve trigeminal branches is the cause of the malformations.

Value of systematic post mortem radiographic examinations of fetuses--400 cases.

A retrospective study of 400 cases of fetal deaths has been carried out to assess the value of systematic post mortem radiological examination. Apart from general diagnosis purpose, special attention was given to the assessment of bone age and mineralization. The results were correlated with the clinical, U.S., chromosomal and pathological data. Computerized analysis of our information show the following results: (1) The radiological examination was valuable for the final diagnosis in 13.5% of cases. (2) It brings additional information in 34.5% of cases. (3) It had no diagnostic value in 52%. Furthermore, several points deserve attention such as apparition of teeth (21 weeks), calcaneum (24 weeks). Major osteoporosis was always associated with a constitutional bone disease or an infectious process. An excessive length of the upper limbs (12) was seen in 11 cases of anencephaly. We suggest that a radiological examination should not be routinely performed, when the diagnosis is otherwise obvious, but should be considered in the presence of dwarfism, or other limb abnormalities and when the gestational age is uncertain. The films provide essential information especially for further genetic counselling. Systematic post mortem radiographic examinations are performed in all naturally aborted fetuses over 11 weeks of gestation in our institution. A retrospective study of the last 400 cases was designed in order to assess the value of this examination, with regard to overall diagnosis and assessment of bone maturation and mineralisation.