Primitive pituitary perivascascular epithelioid cell tumor: A challenging diagnosis of melanocytic pituitary lesion.

BACKGROUND: Perivascular Epithelioid cell tumors (PEComa) are rare mesenchymal tumors. They generally occur in the gynecologic or digestive tract. The diagnosis of Central Nervous System PEComa is exceptional and challenging. CASE DESCRIPTION: We report the case of a 46-year-old woman, with no particular medical history, who presented a secondary amenorrhea and a slight hyperprolactinemia. She was diagnosed on MRI with a pituitary tumor showing spontaneous hypersignal in T1-weighted images. After failure of medical treatment with cabergoline, surgical resection was required due to progressive tumor growth. Macroscopic aspect and initial immunohistochemical features were in favor of a primitive hypophyseal melanocytoma. However, molecular and transcriptional study through targeted exome- and RNA-sequencing led to the exceptional diagnosis of pituitary Perivascular Epithelioid Cell Tumor (PEComa). Three-years of postoperative radio-clinical follow-up showed an asymptomatic non-evolutive small remnant. CONCLUSION: PEComa is an exceptional diagnosis among pituitary tumors. It should be evoked as a potential differential diagnosis in case of primitive melanocytic lesion of the pituitary gland. Specific molecular analysis is mandatory to confirm the diagnosis and exclude differential diagnosis.

Pituitary metastasis of malignant melanoma misdiagnosed as pituitary adenoma: A case report and systematic review of the literature.

We report a case of malignant melanoma revealed by a metastasis to the pituitary gland. The tumor was misdiagnosed as a pituitary adenoma and aggressive transsphenoidal surgery was complicated by a cerebrospinal fluid fistula. Nine weeks later, the patient presented multiple leptomeningeal and brain metastases spreading from the sellar region. Regarding these observations, we conducted a systematic review of the literature in order to investigate clinicoradiological features that should lead clinicians to suspect pituitary metastasis and how it should impact the surgical management.

Head and neck carcinoma of unknown primary.

In adult cervicofacial pathology, carcinoma of unknown primary is defined as lymph-node metastasis the anatomic origin of which is not known at the time of initial management. It constitutes up to 5% of head and neck cancers. Presentation may suggest benign pathology, delaying and confusing oncologic treatment. Diagnostic strategy in cervical lymph node with suspicion of neoplasia requires exhaustive work-up to diagnose malignancy and, in 45% to 80% of cases, depending on the series, to identify the primary site. Histologic types comprise squamous cell carcinoma, thyroid carcinoma, adenocarcinoma, neuroendocrine carcinoma and undifferentiated carcinoma. Association is sometimes found with human papilloma virus or Epstein Barr virus, guiding treatment. The objective of the present study was to provide clinicians with the necessary diagnostic tools, based on the current state of clinical, imaging and pathologic knowledge, and to detail treatment options.

Endoscopic transnasal transseptal pituitary surgery.

Pituitary surgery is performed via a transsphenoidal approach in the vast majority of cases according to various methods that have changed over the years. A microscopic transseptal approach via a sublabial mucosal incision or a nasal mucosal incision has also been extensively used. An endoscopic transnasal approach was first described in the 1990's, followed by the concept of a microscopic transseptal approach and an endoscopic strictly endonasal approach. We use an entirely endoscopic transseptal transsphenoidal approach via an incision in the nasal mucosa for both access and tumour resection. This procedure has a number of advantages: strictly midline approach to the sella turcica, large operative field, no interference between instruments and a low rate of nasal complications.

Direct identification of molds by sequence analysis in fungal chronic rhinosinusitis.

Fungi are widely implicated in chronic rhinosinusitis. Direct microscopic examination (DME) is used to confirm the biological diagnosis of fungal rhinosinusitis (FRS). Diagnostic sensitivity of DME is better than culture, however DME does not allow fungal species identification. In this study, we included 54 sinus samples demonstrating hyphae on DME. Direct sequencing was compared to culture for the identification of the fungal species. Sequence analysis identified fungi in 81.5% of cases while culture was positive in only 31.5%. The most common genus was Aspergillus and the identified species belonged to section Fumigati or to section Flavi. Among other fungi identified by sequence analysis, Schizophyllum commune was present in three samples attesting to the importance of this Basidiomycetes in FRS. Our results clearly demonstrate the superiority of sequencing compared to culture when performed on specimens with hyphal elements at DME, and contributes to the epidemiological knowledge of fungi involved in FRS.

Specific immunotherapy in allergic rhinitis.

Allergic rhinitis is a common condition, with significant impact on quality of life depending on severity and quality of control. Allergen-specific immunotherapy (allergen-SIT) is the only known treatment able to alter the natural course of allergic rhinitis. Although well known to allergologists, it has yet to be fully adopted by the ENT community. This review, based on the most recent meta-analyses and clinical studies, shows that SIT significantly reduces symptoms and medication requirements (nasal corticosteroids, H1-antihistamines) in allergic rhinitis. It can reduce the risk of progression to asthma and, if initiated early enough, of developing new sensitizations. Immunobiological analysis shows an altered inflammatory profile following SIT, with immune tolerance involving T-regulatory lymphocyte induction and IgG production. Sublingual SIT with drops is as effective as subcutaneous SIT and is simpler to use, with less anaphylactic risk. Standardization of trial protocols in terms of treatment response assessment and side effect grading is recommended to improve comparative studies. Sublingual SIT with tablets has recently been introduced, providing a good opportunity for ENT practitioners to adopt the SIT approach in rhinitis triggered by allergy to pollens and, in the near future, to house dust mites.

Guidelines of the French Society of Otorhinolaryngology (SFORL) (short version). Specific treatment of epistaxis in Rendu-Osler-Weber disease.

OBJECTIVES: The authors present the guidelines of the French Oto-Rhino-Laryngology - Head and Neck Surgery Society (Société Française d'Oto-Rhino-Laryngologie et de Chirurgie de la Face et du Cou: SFORL) concerning specific treatment of epistaxis in Rendu-Osler-Weber disease. METHODS: A multidisciplinary work-group was entrusted with a review of the scientific literature on the above topic. Guidelines were drawn up, based on the articles retrieved and the group members' individual experience. They were then read over by an editorial group independent of the work group. The final version was established in a coordination meeting. The guidelines were graded as A, B, C or expert opinion, by decreasing level of evidence. RESULTS: Rendu-Osler-Weber disease is diagnosed from the presence of at least three of Curaçao's four criteria. In acute epistaxis, bidigital compression is recommended. Embolization is reserved for resistant epistaxis. Non-resorbable nasal packing and cauterization are contraindicated. Patient education is essential. Telangiectasia of the nasal mucosa can be treated by various local means. In the event of insufficient control, systemic administration of tranexamic acid is recommended.

Guidelines of the French Society of Otorhinolaryngology (SFORL). Managing epistaxis under coagulation disorder due to antithrombotic therapy.

OBJECTIVE: The authors present the guidelines of the French Society of Otorhinolaryngology concerning the management of epistaxis during antithrombotic therapy. METHODS: A review of the literature was performed by a multidisciplinary work group. Guidelines were drafted, then re-edited by a reading group independent of the work group to produce the final text. The proposed recommendations were graded A, B, C or expert opinion, on decreasing levels of evidence. RESULTS: Before any decision to modify antithrombotic treatment, it is recommended to screen for overdose and assess the risk of thrombosis. In stented patients, dual antiplatelet therapy must be maintained during the month following stenting and, if possible, for 3 months. In epistaxis with antivitamin K (AVK) overdose controlled by packing, corrective measures are based on the International Normalized Ratio (INR). In uncontrolled epistaxis, it is recommended to stop AVK, administer antidotes and regularly monitor INR. In case of intravascular embolization, it is not recommended to alter anticoagulant treatment.

Guidelines of the French Society of Otorhinolaryngology (SFORL). Epistaxis and high blood pressure.

OBJECTIVES: The authors present the guidelines of the French Oto-Rhino-Laryngology - Head and Neck Surgery Society (Société Française d'Oto-Rhino-Laryngologie et de Chirurgie de la Face et du Cou: SFORL) on epistaxis in high blood pressure. METHODS: A multidisciplinary work group was entrusted with a review of the scientific literature on the above topic. Guidelines were drawn up, based on the articles retrieved and the group members' individual experience. They were then read over by an editorial group independent of the work group. The final version was established in a coordination meeting. The guidelines were graded as A, B, C or expert opinion, by decreasing level of evidence. RESULTS: It is recommended to measure the blood pressure of patients in acute-phase epistaxis (Grade A); to control high blood pressure medically in the acute phase of bleeding, to reduce its duration; to monitor blood pressure at the waning of nosebleed; and to control high blood pressure medically in the waning phase to reduce the risk of recurrence. In case of persistent high blood pressure on waning of severe epistaxis, it is recommended to prescribe cardiovascular evaluation to screen for underlying hypertensive disease (Grade B).

Guidelines of the French Society of Otorhinolaryngology (SFORL). Second-line treatment of epistaxis in adults.

OBJECTIVES: The authors present the guidelines of the French Oto-Rhino-Laryngology - Head and Neck Surgery Society (Société Française d'Oto-Rhino-Laryngologie et de Chirurgie de la Face et du Cou: SFORL) on second-line treatment of epistaxis in adults, after failure of anterior and/or anterior-posterior nasal packing. METHODS: A multidisciplinary work group was entrusted with a review of the scientific literature on the above topic. Guidelines were drawn up, based on the articles retrieved and the group members' individual experience. They were then read over by an editorial group independent of the work group. The final version was established in a coordination meeting. The guidelines were graded as A, B, C or expert opinion, by decreasing level of evidence. RESULTS: Arterial embolization should be performed by an experienced interventional neuroradiologist with adequate technical facilities, to reduce the risk of complications. Cerebral and supra-aortic vessel CT angiography should be performed in case of post-traumatic epistaxis with suspected internal carotid injury. In case of persistent bleeding despite endoscopic hemostasis of the sphenopalatine artery, anterior ethmoidal artery hemostasis should be performed via a medial canthal incision, with endoscopic assistance as needed. In case of persistent epistaxis despite the usual surgical and neuroradiological procedures, surgical exploration of the sinonasal cavities should be performed, with elective coagulation in case of bleeding from secondary branches, and/or ethmoidectomy in case of diffuse bleeding. A decision-tree was drawn up for the management of second-line treatment of epistaxis.

Guidelines of the French Society of Otorhinolaryngology (SFORL). First-line treatment of epistaxis in adults.

OBJECTIVES: The authors present the guidelines of the French Otorhinolaryngology-Head and Neck Surgery Society (SFORL) on first-line treatment of epistaxis in adults. METHODS: A multidisciplinary work-group was entrusted with a review of the scientific literature on the above topic. Guidelines were drawn up, based on the articles retrieved and the group members' individual experience. They were then read over by an editorial group independent of the work-group. The guidelines were graded as A, B, C or expert opinion, by decreasing level of evidence. RESULTS: In first-line, clearing out blood-clots and bidigital compression are recommended. In case of persistent bleeding, local anesthesia with a vasoconstrictor is essential before nasal diagnostic and therapeutic procedures. When the origin of bleeding is not anterior, nasal endoscopy is an essential procedure, identifying the bleeding site in most cases. In case of active bleeding, cauterization is recommended but is only feasible if the bleeding site is clearly visible. When the bleeding site is not identifiable or the first measures failed, anterior packing may be performed by a non-specialist physician. Epistaxis requires subsequent nasal endoscopy performed by an ENT specialist. Patients should be informed of the measures to be taken in case of epistaxis at home, and the risks associated with the various treatments.

Gardner's syndrome presenting with a fibromatous tumour of the parotid.

INTRODUCTION: Gardner's syndrome is the association of familial adenomatous polyposis (FAP) with an anaphase promoting complex (APC) gene mutation and several extradigestive manifestations: osteomas, epidermal cysts and desmoid tumours. Only 2 cases of FAP associated with parotid tumour have been reported in the literature: one carcinoma and one pleomorphic adenoma. CASE REPORT: We report the case of a 23-year-old man with Gardner's syndrome presenting with a fibromatous tumour of the left parotid gland. The other clinical manifestations were an osteoma of the skull base and a mesentery desmoid tumour. Left total parotidectomy was performed, followed by gastroscopy and colonoscopy, demonstrating numerous colonic adenomas. Genetic analysis revealed an APC gene mutation, confirming the diagnosis. Total prophylactic colectomy was performed. CONCLUSIONS: This is the first reported case of Gardner's syndrome associated with a fibromatous tumour of the parotid. Early management of FAP is essential to prevent colorectal cancer that occurs in 100% of untreated cases. The rarity of this syndrome and the parotid site can lead to delayed diagnosis.

Interest of video-assisted minimally invasive surgery in primary hyperparathyroidism.

INTRODUCTION: Surgery is the only radical and definitive treatment for primary hyperparathyroidism. Exploration of the four parathyroid sites is giving way to minimally invasive techniques. The present study sought to compare two minimally invasive parathyroidectomy techniques, by classical cervicotomy (MIP-C) and by video-assistance (MIP-VA), in terms of success rate, complications rate, operating time, and patient and community physician satisfaction. MATERIALS AND METHOD: A non-randomized retrospective comparative study included 112 patients presenting with primary hyperparathyroidism with identified parathyroid adenoma, operated on between January 2005 and October 2010. The two groups were constituted according to the surgeons' habitual practice: 54 cases of MIP-VA and 58 of MIP-C. RESULTS: Results for MIP-VA and MIP-C were respectively: success, 96.3% vs. 100% (P=0.09); mean scar size, 1.47 vs. 3.43cm (P<0.01); hypocalcemia, 2 vs. 3 cases (P=0.1); theater time, 94.25 vs. 76min (P=0.02); and postoperative stay, 1.08 vs. 1.37 days (P=0.07). Patient satisfaction was comparable between groups, while 93.3% of community physicians found MIP-VA preferable to MIP-C, although only 39.3% had known the MIP-VA technique. CONCLUSION: With efficacy, morbidity and patient satisfaction comparable to classical surgery, MIP-VA significantly reduced cervicotomy size and hospital stay. Community physicians considered it to be preferable to MIP-C.

Robotic endoscopic sinus and skull base surgery: review of the literature and future prospects.

OBJECTIVE: There has been a considerable growth in the indications of endonasal surgery that now include malignant tumours of the nasal fossae and anterior and middle cranial fossa. However, new limitations have also been identified, such as bleeding and cerebrospinal fluid leak, as well as the need to use several instruments simultaneously. Can robotics provide solutions to these problems? METHOD: Review of the literature based on the three main databases: Medline, Pubmed and Cochrane. RESULTS: Ten publications were identified. Some authors have developed surgical approaches to the skull base using the da Vinci(®) robot, while others have designed specific robots. CONCLUSION: None of the currently available solutions appears to be completely suitable. The da Vinci(®) robot is very cumbersome and can only be used in the middle cranial fossa via complex and relatively invasive routes. The other robots are laboratory prototypes. We are currently developing an innovative, compact, ergonomic and safe dedicated endoscope holder.

Positron emission tomography-computed tomography evaluation for recurrent differentiated thyroid carcinoma.

OBJECTIVES: Recurrence is observed in 15-20% of patients under surveillance following treatment of differentiated thyroid cancer (DTC). However, due to cell dedifferentiation, the recurrence may be iodine-negative, thereby compromising detection. For this reason, new methods of exploration are indispensable to enable localization of such recurrences. The purpose of this work is to review the contribution of positron emission tomography-computed tomography (PET-CT) in the exploration of iodine-negative recurrent DTC. METHOD: A comprehensive review and discussion of the medical literature was carried out. RESULTS: Depending on the report, the sensitivity of PET-CT ranged from 70% to 85%, with up to 90% specificity. However, the large number of false negatives, which can reach 40%, is the disadvantage of this examination. PET-CT results lead to change in the therapeutic strategy in approximately 50% of patients with isolated raised serum thyroglobulin levels, and surgical exploration of a precise anatomical area in the neck. CONCLUSION: As post-treatment recurrence of a DTC can affect patient survival, a thorough diagnostic work-up is required in these cases. Where thyroglobulin levels are elevated with no uptake on 131-iodine scans, PET-CT can be a useful complementary exploration, especially for localizing the site of recurrence.

Cervical lymphadenopathies signaling thyroid microcarcinoma. Case study and review of the literature.

BACKGROUND: Some lateral cervical lymphadenopathies may lead to the discovery of papillary microcarcinomas (PMC) of the thyroid that are not radiologically apparent. This relatively rare clinical situation raises questions about the diagnostic approach to chronic cervical lymphadenopathy and the impact of lymph node metastasis on PMC prognosis. PURPOSE OF THE ARTICLE: To study the epidemiologic, clinical, and prognostic criteria of cases of lymphadenopathy that signaled PMC. PATIENTS AND METHODS: A retrospective study of 167 consecutive cases of PMC compared with 13 cases where a cervical mass signaled other forms of PMC. RESULTS: The mean age was 48.5 years, the ratio of men to women was 5:8, and the mean PMC size was 5.5mm. These data did not differently significantly from those of the other PMC cases. The preoperative imaging found fluid content in six cases, with microcalcifications in three cases. All cases were treated by modified radical neck dissection on the side with the lymphadenopathy and total thyroidectomy with central neck dissection. The lymphadenopathy included a ruptured capsule in five cases and was accompanied by central lymph node metastases in three cases. Thyroid capsule involvement was significantly more common in cases of PMC discovered due to lymphadenopathy than in other cases of PMC (69% versus 9.7%, respectively; p<0.001). The mean follow-up was 7.3 years. There were no deaths due to PMC signaled by lymphadenopathy. Two cases of lymph node recurrence after 8 and 10 years were controlled by another surgery and radioactive iodine treatment. CONCLUSION: Any chronic cervical mass should suggest the possibility of thyroid origin, especially in cases with cystic content or microcalcifications in subjects with no particular risk factors. An ultrasound of the thyroid should be done, as well as a fine needle aspiration biopsy of the lymphadenopathy with a thyroglobulin assay. Treatment is the same as for any thyroid carcinoma, and results in a good oncological outcome, despite the possibility of lymph node recurrences.

Pemphigus vulgaris antigen mRNA quantification for the staging of sentinel lymph nodes in head and neck cancer.

BACKGROUND: Molecular diagnosis has been proposed to enhance the intra-operative diagnosis of sentinel lymph node (SLN) invasion in head and neck squamous cell carcinoma (HNSCC). Although cytokeratin (CK) mRNA quantification with real-time reverse transcriptase-PCR (QRT-PCR) has produced encouraging results, the more discriminating markers remain to be identified. METHODS: Pemphigus vulgaris antigen (PVA), squamous cell carcinoma antigen (SCCA), and CK17 mRNA were quantified using QRT-PCR, and the results were compared with an extensive histopathological examination of the entire SLNs on 78 SLNs harvested from 22 patients with HNSCC. RESULTS: SCCA and CK17 quantification showed significantly higher mRNA values for macrometastases (MAs) than for either negative or isolated tumour cell (ITC) SLNs (P<0.01). Pemphigus vulgaris antigen allowed the discrimination of all MAs and micrometastases from both negative and ITC SLNs (P<0.001). For the neck staging of patients, considering metastatic vs non-metastatic status, receiver-operating characteristic curve analysis found areas under the curve of 93.8, 97.9, and 100% for CK17, SCCA, and PVA, respectively. With PVA, a cutoff value of 562 copies per 100 ng of cDNA permitted the correct distinction between patients with positive as opposed to negative neck nodes in all cases. CONCLUSION: PVA seems to be a highly promising marker for accurate intra-operative SLN staging in HNSCC by QRT-PCR.

[Sinonasal adenocarcinomas reviewed. Prognostic value of WHO 2005 histological classification]. / Les adénocarcinomes nasosinusiens revisités. Intérêt pronostique de la classification histologique OMS 2005.

OBJECTIVES: The WHO 2005 histological classification separates sinonasal adenocarcinoma (ADC) into three classes: intestinal-type adenocarcinoma (ITAC), low-grade sinonasal ADC and high-grade sinonasal ADC. The goal of this study was to check the relevance of this classification on the prognosis of patients treated for ADC. PATIENTS AND METHODS: All the files of patients treated consecutively in the ENT department of the Montpellier University Hospital for ADC between 1980 and 2003 were retrospectively re-examined. Each case was reviewed for anatomical and pathological data based on the immunohistochemistry results according to the WHO 2005 classification, with a study of a panel of markers: cytokeratin 7 (CK7), cytokeratin 20 (CK20), Villin, CDX2 and EGFR. The epidemiologic data, the methods of treatment and the follow-up were studied. The survival probabilities were calculated using the Kaplan-Meier method and the survival graphs were compared using a log-rank test. RESULTS: Sixty-two files were reviewed. Twelve patients were reclassified into the adenoid cystic carcinoma category and excluded from the study. In the 50 remaining cases, there were 36 ITAC cases, four low-grade ADC cases and 10 high-grade dedifferentiated carcinomas. For all of the ADC cases, the total survival at 5 years and without recurrence was 64 and 52%, respectively. The analysis of the three subgroups showed a total survival of 72.2% for ITAC, 100% for low-grade and 20% for high-grade ADC with a significant difference (p=0.044). This immunohistochemical distinction was mainly based on the expression of CK20 found in 98% of the ITAC cases and absent in low- and high-grade ADC patients. CONCLUSION: The WHO 2005 classification for sinonasal ADC provides a valuable prognosis by showing a difference in the progression profile between ITAC, low-grade ADC and high-grade ADC. Moreover, broader studies should be conducted to investigate the different subtypes of ITAC.

[Study of the platysma coli muscle vascularisation by the facial artery. Implication during the elevation of the musculo-cutaneous platysma coli muscle flap]. / Etude de la vascularisation du muscle platysma coli par les branches de l'artère faciale. Implications lors du prélèvement du lambeau musculo-cutané de platysma coli.

AIM OF THE STUDY: The purpose of this study was to assess the vascularity of the platysma muscle by the branches of the facial artery, in order to determine the best means of harvesting a musculo-cutaneous flap while ensuring maximum vascular security. PATIENTS AND METHODS: Ten platysma muscles were dissected on 4 fresh specimens and one formaldehyde-preserved specimen. The dissection was performed after injection of the facial artery in 6 cases, while 4 muscles were dissected without any previous injection. RESULTS: The vascular supply of the platysma muscle comes essentially from the branches of the submental artery and from branches descending straight from the facial artery. Other collateral branches contribute to this vascularization, but their importance is minor. All these arteries reach the muscle, entering its visceral aspect, then proceed to the sternal notch in a radial axis. CONCLUSION: The size of the flap has to be defined within a quadrilateral figure with its base formed by the mandibular edge and its apex by the inferior limit of the flap. It is essential to preserve the maximum possible muscular thickness, especially on the medial side of the flap. If the facial artery needs to be ligated, this has to be done as it enters the submandibular space in order to protect most of the collateral branches destined to the muscle. The vascularization is then taken back by the homo- and contro-lateral facial vascularisation in an inverted flow in the remaining segment of the facial artery.