Primary Endoscopic Management of Apoplexy in a Giant Pituitary Adenoma.

BACKGROUND: Sellar lesions with large suprasellar extension represent a neurosurgical challenge because of their nature and anatomic complexity. The goal of the extended transphenoidal-transtuberculum approach is enlarging the transsphenoidal route superiorly and laterally allowing for a surgical adequate exposure and offering a remarkable versatility in many sellar pathologies. CASE DESCRIPTION: We present the case of a 65-year-old man who suddenly developed blindness, right hemiparesis, and decreased alertness. The initial head computed tomography scan revealed a pituitary apoplexy of a giant adenoma associated with hydrocephalus resulting from obstruction of the foramen of Monro. The video shows a complete lesion removal through the sole endoscopic approach, with opening of the dural layer of sphenoidal plane and successful decompression of the third ventricle. Visual and functional improvement occurred in the immediate postoperative course. No lumbar drain has been used. CONCLUSIONS: This case demonstrates how the endoscopic approach can be attempted as a first and possibly stand-alone option for the surgical management of large sellar-suprasellar lesions. The endoscopic route is not associated with high rates of major complications and is safe when performed by experienced surgeons. In fact, it guarantees an enhanced control of the vascular feeders reaching the tumor from the anterior and middle fossa and results in a satisfactory manipulation of lesions invaginating into the floor of the third ventricle. A careful preoperative assessment of Knosp grade, tumor volume, hemorrhagic components, suprasellar extension, and sphenoid sinus invasion should always guide the management plan and suggest a staged or a combined (with transventricular or pterional approach) removal in particularly challenging cases.

Isolated fronto-ethmoidal allergic fungal rhinosinusitis: case report and review of the literature.

BACKGROUND AND AIM OF THE WORK: Fungal rhinosinusitis (FRS) is a clinical entity characterized by the presence of fungi within sino-nasal cavities that may occur in patients with normal or defective immunity. Allergic fungal rhinosinusitis (AFRS) is a form of non-invasive FRS that affects patients with an abnormal immuno-mediated response to fungal antigens. This article describes a case of isolated fronto-ethmoidal AFRS. METHODS: A 20-year old male patient presented with a history of a left nasal respiratory obstruction and allergic oculorhinitis. CT scans showed a polypoid mass in the left nasal cavity and opacification of the left ethmoid sinus, frontal recess and frontal sinus with hyperdense component. The patient underwent functional endonasal sinus surgery (FESS) with removal of nasal polyps from the left nasal cavity and of cheesy-like material and dense mucus from the left ethmoid and frontal sinus. Histological examination showed presence of fungal hyphae within the allergic mucus; a diagnosis of AFRS was made. RESULTS: Follow up at 14 months showed no signs of recurrence. CONCLUSIONS: The AFRS case reported herein is characterized by isolated unilateral fronto-ethmoid involvement, a rare presentation. Endoscopic nasal treatment was effective with complete patient recovery. (www.actabiomedica.it).

First Case of Primary Sellar/Suprasellar-Intraventricular Ewing Sarcoma: Case Report and Review of the Literature.

BACKGROUND: Intracranial Ewing sarcoma (ES) and peripheral primitive neuroectodermal tumors (pPNETs) are extremely rare and poorly differentiated neoplasms. Immunohistochemical and cytogenetic findings support the possibility of a unique nosologic entity. Primary intracranial localization of this tumor is extremely rare; a few cases are reported in the literature, with only some confirmed by genetic studies. CASE DESCRIPTION: We report a 12-year-old patient with a sellar/suprasellar mass with intraventricular extension that in all its features mimicked a transinfundibular craniopharyngioma. The patient underwent complete resection of the lesion via an endoscopic endonasal transtuberculum approach 6 days after ventriculoperitoneal shunt for acute obstructive hydrocephalus. Histopathologic and genetic examination demonstrated ES/pPNET. The diagnosis was confirmed by detection of a rearrangement of the EWSR1 gene by fluorescent in situ hybridization and identification of the diagnostic t(11;22) translocation by reverse transcriptase polymerase chain reaction. The patient remained in complete clinical remission 12 months after tumor resection followed by adjuvant chemotherapy with no radiologic evidence of tumor recurrence. CONCLUSIONS: To our knowledge, this is the first case of primary intrasellar/suprasellar-intraventricular ES/pPNET confirmed by molecular genetic analysis. Extensive investigations, including pathologic, immunohistochemical, and genetic studies, are needed for differentiation of these tumors from other, more common sellar/suprasellar tumors. Our case highlights that an interdisciplinary therapeutic approach is mandatory to guarantee a favorable outcome.

Occult nasolacrimal duct ectasia in a child with acute sinusitis: clinicopathological considerations and literature review.

BACKGROUND: Enlargement of the nasolacrimal duct (NLD) in the absence of neoplasm is rare. As there are few reports on this condition in the literature, its pathologic significance and proper management remain unclear. We report a case of asymptomatic NLD enlargement incidentally discovered on high resolution computed tomography scan performed for a complicated acute sinusitis. METHODOLOGY: Case report and literature review. RESULTS AND CONCLUSIONS: NLD enlargement, defined as a truly isolated asymptomatic dilatation of the NLD, is rarely encountered. Etiology and clinical significance are unclear although the absence of nasolacrimal pathway or sinonasal disease and radiological findings of bone remodelling suggest that it may be congenital. In such cases, diagnosis may be incidental and based on imaging studies, and the term NLD ectasia seems to be more appropriate to describe this distinct entity. NLD enlargement may favour osteomeatal complex obstruction, thus leading to severe acute sinusitis.

Pyogenic spondylodiscitis and osteoradionecrosis of the cervico-thoracic spine: a rare complication after surgery and radiation for hypopharyngeal cancer.

OBJECTIVE: To describe a rare case of osteoradionecrosis and pyogenic spondylodiscitis of the cervico-thoracic spine after surgery and radiotherapy for hypopharyngeal cancer. METHODOLOGY: Diagnostic clues are presented and critically discussed with particular regard to computed tomography, magnetic resonance and positron emission tomography features advising the association between and osteoradionecrosis and spondylodiscitis. RESULTS AND CONCLUSIONS: Pathogen isolation via CT-guided biopsy provided the definite diagnosis and indicated the targeted therapy. Successful recovery was obtained with conservative treatment.

Congenital nasal pyriform aperture stenosis: diagnosis and management.

BACKGROUND: Congenital nasal pyriform aperture stenosis is a rare and potentially lethal form of airway obstruction in newborns. Immediate recognition and appropriate therapy are mandatory for this potentially life-threating condition. This anomaly may present as an isolated malformation or may be associated with other cranial-facial anomalies. Clinically, CNPS shows unspecific symptoms of nasal airway obstruction such as apnoic crisis, episodic cyanosis and inability to nurse. The purpose of this report is to present author's experience in the surgical management of this rare pathology in 3 patients. PATIENTS AND METHODS: Three cases of congenital nasal pyriform aperture stenosis were reviewed for presentation of the disorder, management and effectiveness of the surgical treatment. RESULTS: All the patients underwent a surgical correction of the pyriform aperture stenosis using a sublabial approach followed by nasal stenting. During the follow-up no cases of restenosis, respiratory failure or cyanosis were detected. CONCLUSIONS: Congenital pyriform aperture stenosis should be suspected in newborns with clinical signs of severe nasal obstruction associated with a difficulty to pass a small catheter though the anterior nasal valve. Timely recognition is mandatory to prevent a potential deadly outcome. Surgical correction of the stenosis though a sublabial approach followed by a nasal stenting revealed to be most effective treatment for these patients.

Prophylactic central neck dissection for papillary thyroid carcinoma: the terms of the debate.

An increasing interest in the management of central neck lymph nodes in the surgical treatment of papillary thyroid cancer is observed. While is widely accepted that patients with clinically apparent lymph nodes metastases should undergo compartment-oriented neck dissection, controversy exists about the need for prophylactic central neck dissection. The main issues that have been raised are the following ones: the definition of the anatomic boundaries and terminology of central neck dissection, the value of the recommendations expressed by the most authoritative association, the arguments in favour and against the appropriateness of prophylactic neck dissection, the laterality of central neck dissection. This article aims at reviewing the literature on prophylactic central neck dissection for papillary thyroid cancer in order to clarify some issues and to offer the reader a clear and concise overview of this complex debate.

Clinical outcomes of rhabdomyosarcoma and Ewing's sarcoma of the head and neck in children.

OBJECTIVE: To review our experience and critically evaluate treatment strategy and results in children with head and neck rhabdomyosarcoma and Ewing's sarcoma. METHODS: Retrospective charts review of children affected by non-orbital rhabdomyosarcoma or Ewing's sarcoma of the head and neck who were treated at our institution from January 1996 to August 2009. RESULTS: Seven consecutive children with head and neck rhabdomyosarcoma or Ewing's sarcoma were identified. Four children had rhabdomyosarcoma, 3 children had Ewing's sarcoma. Regions involved were: cheek, ethmoid and maxillary sinuses, nasopharynx, middle ear/mastoid and frontal bone. In one case, surgery was performed as primary treatment modality; the other children were treated firstly with chemotherapy. Three patients underwent surgical resection after chemotherapy, while 4 patients received radiotherapy. Five children are disease free after a median of 7.7 years from initial diagnosis. Two patients relapsed after 10 and 29 months from initial diagnosis respectively; despite the administration of additional therapy both children died of disease. CONCLUSION: Treatment for rhabdomyosarcoma and Ewing's sarcoma consists in a multimodal therapy involving chemotherapy, radiotherapy and surgery. The optimum use, timing and intensity of these three treatments are still matters of international debate. Chemotherapy in association with radiotherapy has proven capable to obtain local and distant control of disease. But when surgery is unfeasible or fails in radicality, local control is difficult without radiotherapy. Despite additional therapeutic efforts, prognosis of relapsing disease remains poor.

Simultaneous management of the enophthalmos and sinus pathology in silent sinus syndrome: a report of three cases.

Silent sinus syndrome is a rare disorder affecting the maxillary sinus unilaterally, characterized by ipsilateral enophthalmos and hypoglobus. The treatment is surgical: the endoscopic approach represents the gold standard for the restoration of normal sinus aeration, but there is no consensus in the management of the enophthalmos. Most authors suggest a two stage procedure consisting of endoscopic sinus surgery initially and a delayed secondary operation for the restoration of the orbital floor. We present our experience with three cases treated with a single-stage procedure, focusing on the advantages of this one-step approach.

Combined endoscopic transsphenoidal-transventricular approach for resection of a giant pituitary macroadenoma.

OBJECTIVES: Sellar lesions, such as pituitary adenomas, even when extended to the suprasellar space may be usually removed through a trans-sphenoidal approach. Larger lesions extending well beyond the edges of the sellar diaphragm such as giant adenomas are best controlled with craniotomy and/or a combined approach that implies both, transphenoidal and transcranial route. Currently, the availability of more sophisticated endoscopes in this type of surgery has provided optimal angles of view and rendered the trans-sphenoidal route less invasive yet, more effective. CASE DESCRIPTION: The authors report a case of a giant pituitary adenoma successfully managed by a simultaneous, combined endoscopic trans-sphenoidal-transventricular approach. CONCLUSION: In selected case of giant pituitary adenoma with ventricular extension, this technique may help to achieve a gross total removal avoiding the need of staged procedures allowing also a direct visualization of the extent of removal. Finally this approach can potentially improve gross total resection rate of different types of tumor involving this region such as cranipharyngiomas while reducing morbidity and mortality.

Involvement of level I neck lymph nodes and submandibular gland in laryngeal and/or hypopharyngeal squamous cell carcinoma.

OBJECTIVE: To investigate the prevalence of level I neck lymph node metastases or submandibular nodal metastases in laryngeal and/or hypopharyngeal squamous cell carcinoma (SCC). PATIENTS AND METHODS: One hundred fifty consecutive neck dissection specimens from 100 patients with laryngeal and hypopharyngeal SCC, who were treated at our institution between 1992 and 2002, were retrospectively reviewed. RESULTS: The tumour stage was T1-T4, and the neck stage was N0-N3. Metastases were never found in level I (Ia + Ib) or in the submandibular gland. Metastases were concentrated within the jugular chain (levels II-IV in 92.2% of the N-positive necks). CONCLUSION: Metastases of level I of the neck and the submandibular gland are extremely rare in cases of laryngeal and/or hypopharyngeal carcinoma. The risk of facial or hypoglossal nerve injury does not justify the dissection of level I and of the submandibular gland in this type of tumour.