RESUMEN

INTRODUCTION AND IMPORTANCE: Primary intracranial neuroendocrine tumors (NETs) are exceedingly rare, often posing diagnostic challenges, particularly in non-secreting variants. These tumors may initially present with nonspecific symptoms, leading to delayed diagnosis and potential neurological complications. CASE REPORT: We present the case of a 33-year-old male admitted with a one-year history of progressively worsening headache accompanied by acute left ptosis and diplopia. Initial examination revealed left eye ptosis and hypotropia, indicative of third and fourth cranial nerve paralysis. Cavoscopy revealed a mild anterior wall bulge of the sphenoid with normal mucosa. MRI imaging unveiled an expansive clival process extending towards the sellar region and left cavernous sinus, completely occupying the sphenoid sinus and exerting mass effect on the pituitary stalk. Hormonal assays were within reference ranges, ruling out a hormonally-active tumor. Endoscopic endonasal surgery for biopsy revealed a low-grade neuroendocrine tumor positive for cytokeratin AE1/AE2, chromogranin A, synaptophysin, and beta-catenin, with a Ki-67-labeling index <2 %. Somatostatin receptor scintigraphy confirmed intense hyper fixation of the tracer in the sphenoidal tumor, supporting its neuroendocrine origin. The patient declined surgical intervention after informed consent, opting for C1 somatostatin analogs prior to radiotherapy. One-year follow-up demonstrated symptom stability with no tumor progression. CLINICAL DISCUSSION: To this day, no consensus among reports concerning the optimal management of these cases. Imaging assessment is crucial to validate the primary nature of the tumor and to exclude any distant localization. Various therapeutic modalities, such as surgery, radiotherapy, and somatostatin analogs, should be considered based on the specific characteristics and extent of the tumor. CONCLUSION: Our case is a clear reminder that neuroendocrine tumors should be considered as a differential diagnosis for skull base neoplasms.

RESUMEN

Our study of the most representative case series of children and teenagers with thyroid cancer in Morocco (22 cases) aimed to highlight the characteristics of this cancer among children and teenagers and to compare results with those in the international literature. We conducted a retrospective, descriptive study of patients with differentiated thyroid cancer, hospitalized in the Department of Otolaryngology-Head and Neck Surgery at the Hospital in Casablanca, Morocco, over the period January 1995-March 2015. We collected data about 22 cases that met our inclusion criteria. The average age of our patients was 14 years, sex-ratio was 3.4, most of our patients presented with thyroid nodule associated, in 22.7% of cases, with cervical lymphadenopathy and in 9.1% of cases with signs of compression. All patients underwent total thyroidectomy followed by lymph node dissection in 31.82% of cases. The diagnosis of thyroid cancer was based on anatomo-pathological examination of the surgical specimen which confirmed the diagnosis of papillary carcinoma in 95.4% of cases and of vesicular carcinoma in 4.5% of cases. 100% of cases received I-131 RAI treatment followed by thyroid hormone replacement therapy. Close supervision and regular monitoring enabled the detection of nodal metastasis in 3 patients and of distance metastases in 4 patients. Differentiated thyroid cancer among children and teenagers is rare but aggressive, its treatment is based on surgery associated with iratherapy enabling excellent prognosis.

Asunto(s)

RESUMEN

BACKGROUND: Isolated sphenoidal sinusitis is an uncommon cause of headaches in children and adolescents. Recognizing the condition on physical examination alone can be challenging, and delayed diagnosis often occurs. CASE PRESENTATION: A 4-year-old child presented with symptoms of headache, fever, and vomiting. Nasal endoscopy, computed tomography (CT), and magnetic resonance imaging (MRI) were used to confirm the diagnosis of isolated sphenoiditis. The patient was treated with antibiotics and steroids. However, the patient developed meningoencephalitis as a complication of the untreated isolated sphenoiditis. CONCLUSION: Isolated sphenoidal sinusitis can lead to serious complications if left untreated. Adjunctive imaging and prompt treatment are essential to prevent such complications in children and adolescents. This case highlights the importance of considering isolated sphenoiditis in the differential diagnosis of pediatric headaches and the need for early diagnosis and treatment.

Asunto(s)

RESUMEN

Squamous cell carcinoma (SCC) of the auricle is a rare and aggressive entity of cell carcinomas. It is mostly identified in older males with history of sun exposure. After histopathological confirmation, the initial assessment which consists of clinical and radiological evaluation will determine the therapeutic strategy. We report the case of a neglected SCC of the left pinna with parotid and temporo-mandibular infiltration. After surgical resection, the patient underwent a two staged reconstructive surgery. This was followed later on with radiotherapy and chemotherapy. The evolution was favourable for our patient during an 18 months follow-up. This case report underlines the importance of both curative and reconstructive surgery in successfully treating locally advanced tumors of the temporal bone.

RESUMEN

Hearing loss can result from a wide range of pathologies affecting patients of all ages. It may be due to abnormalities of the outer, middle or inner ear. In this article, we present a case of a 50 years old female patient presenting to the ENT and Head & Neck surgery department of 20 August hospital, complaining of bilateral hearing loss, in whom clinical examination found right attical retraction pocket with scales and left normal tympanic membrane. Complementary investigations revealed the association of two distinct conditions, rarely described in literature: otosclerosis, and chronic otitis media with cholesteatoma in right ear and left otosclerosis. The challenge in this case was to determine the therapeutic strategy: start with left otosclerosis? the right cholesteatoma? simultaneously treat otosclerosis and cholesteatoma right?

RESUMEN

INTRODUCTION: Chondrosarcomas of the head and neck are extremely rare, accounting for approximatively 0.1% of all head and neck malignant tumors. Mesenchymal chondrosarcomas are particularly aggressive, with a high propensity for recurrence. CASE REPORT: We report the case of a 25 years old man with no prior medical history, who presented to our facility with an aggressive tumor of the left maxilla. Upon radiologic and histopathological examinations, the tumor was revealed to be a mesenchymal chondrosarcoma. The patient was treated using surgical excision of the tumor, neoadjuvant chemotherapy and adjuvant radiotherapy. CONCLUSION: Due to the high risk of recurrence and the possibility of metastasis occurring long after the initial diagnosis, it is important that patients with mesenchymal chondrosarcomas receive close and regular follow-ups after treatment.

RESUMEN

INTRODUCTION: The fungal balls of the paranasal sinuses are usually seen in the maxillary and sphenoid sinuses. Although, the lesion of the concha bullosa, without sinus participation, is very uncommon. We report the case of a fungal ball of concha bullosa in an 88-year-old patient. OBJECTIVE: The objective of our review of literature is to investigate the epidemiological, clinical, paraclinical, and therapeutic characteristics of patients diagnosed with fungus ball in concha bullosa. METHODS: A case of a patient who was diagnosed with concha bullosa of a fungus ball is reported. Demographic data, clinical presentation, imaging, and treatments were recorded. Key images were obtained. A review of the literature was also performed. RESULTS: A total of 12 cases have been reported so far in the literature revealed by different symptoms. The mean age was 38.8 years and the gender ratio was ∼12 (female):1 (male). The endoscopic surgical approach was the most frequently used treatment and provides good outcomes. Neither postoperative complications nor recurrences were noted, however, there is insufficient follow-up data. CONCLUSION: Concha bullosa fungal ball is a rare diagnosis that can be revealed by different symptoms. It should be considered in patients with and unexplained chronic facial pain. A preoperative computed tomography scan is an essential tool in making a diagnosis. Endoscopic surgery is the treatment of choice, with a low morbidity and recurrence rate.

RESUMEN

Pilomatrixoma is a rare benign skin tumor differentiating toward hair matrix cells usually encountered in the head and neck region. It is most frequently appearing in the first and second decades of life. Histopathological examination is essential to make definitive diagnosis. Herein, we present an atypical case of multiple pilomatrixomas. A 69-year-old man with multiple voluminous masses over the scalp. Among the three lesions, one was clinically suspicious for malignancy, it measured 17 cm and was ulcerated in places. Histopathology confirmed the diagnosis of pilomatrixoma. The tumors were removed surgically with free margins. Otolaryngologist should be familiar with this benign tumor when evaluating soft-tissue mass in the head and neck region.

RESUMEN

INTRODUCTION: Lipoblastoma is a rare benign tumor arising from embryonic white fat which occurs in the early childhood. It usually arises on the extremities and considered as a rare cause of a pediatric head and neck masses. The aim of this study is to shed light on lipoblastomas as a differential diagnosis of rapidly growing soft fatty masses of children in neck and head area. PATIENTS AND METHODS: A retrospective review of 3 patients with lipoblastoma, underwent Surgical resection (case 1 and 2) by cervical approach. The third patient with a facial lipoblastoma was not operated due to the high risk of facial paralysis. Review of literature, diagnostic methods and genetics of lipomatous tumors are discussed. RESULTS: Complete surgical excision via a cervical approach demonstrated irregular lobules of immature fat cells separated by a loose, myxoid connective tissue. Histology analysis confirmed the diagnosis of lipoblastoma. DISCUSSION: Lipoblastoma is a rare childhood tumor, even rarer in head and neck area. The pathogenesis is unknown, though it is believed to arise from altered embryogenesis of human white fat and genetic predisposition, as chromosome 8 abnormalities may be implicated in the development of lipoblastoma. The presumptive diagnosis is performed by imaging. The most important differential diagnosis of lipoblastoma is myxoid liposarcoma. The mainstay of treatment is complete non-mutilating resection of the tumor to avoid recurrence. CONCLUSION: Lipoblastoma should be suspected in case of heterogeneous fatty tumor in head and neck area, and included as a differential diagnosis of cervical masses in children younger than 3 years. The mainstay of treatment is complete surgical excision with a good prognosis.

RESUMEN

INTRODUCTION: Chronic rhinosinusitis with polyposis (CRSwNP) is a multifactorial naso-sinusal inflammatory disease that affects 2-4% of the adult population. It highly affects the patient quality of life (QoL) in many levels making it a public health issue. The management of CRSwNP is based on a detailed clinical history, a complete endoscopic examination and a precise computed tomographic (CT) analysis. The aim of this study is to evaluate the prevalence and severity of the various CRS clinical manifestations as well as to highlight the potential relationship between symptom scores, asthma and ESS outcomes. PATIENTS AND METHODS: A retrospective cohort study was performed in the 20 August hospital, between January 2017 and December 2018, on patients diagnosed with CRS according to guidelines recommendations, and were beforehand refractory to initial medical therapy and elected to FESS. The patients were divided into two groups, the first group (G1) of patients with asthma and the second (G2) without asthma in order to expose an eventual significant difference in the improvement of symptoms after surgery. The Sino Nasal Outcome Test-22 (SNOT-22) was used to evaluate QOL. RESULTS: A total of 100 patients participated in the study with an average age of 44.53 years. The sex ratio was 1.04 (51% men). Asthma was present in 48% of patients while 20% of patients were intolerant to aspirin with a significant difference between the asthmatic and non-asthmatic group (p < 0.05). It appears that asthma was not objectively correlated with a higher Lund Mackay radiological score (p > 0.05). A higher significant improvement was observed between preoperative and postoperative SNOT-22 scores in group with asthma [42.7 ± 16.3 versus 11.8 ± 9.1] and in group without asthma [38.3 ± 15.1 versus 10.5 ± 14.2]. CONCLUSION: Asthma in CRS is an additional symptom in these patients, mainly reflected in the subset of nasal symptoms in SNOT-22. However, it did not significantly affect the quality of life of the CRSwNP population.

RESUMEN

INTRODUCTION: Maxillary sinus adenoid cystic carcinoma (MSACC) is a rare malignancy with a propensity for distant metastasis CASE PRESENTATION: We report a case of a 55 years-old male who was admitted to our department with a complaint of right nasal obstruction and anosmia. Clinical examination, radiological investigations and histopathological examination found a mass compatible with advanced adenoid cystic carcinoma of the right maxillary sinus. Treatment consisted of radio chemotherapy. CONCLUSION: Adenoid cystic carcinoma of the maxillary sinus may represent a serious diagnostic challenge. It should be considered in the differential diagnosis of Sino nasal tumors even if it's rare.

RESUMEN

Nodular hidradenoma is a rare benign adnexal tumor. It is most frequently encountered in the head and neck region, trunk, and extremities. This tumor exhibits a high recurrence rate, and an association with malignancy.Many names have been used to describe this pathology.We report the case of a nodular hidradenoma in a 30-year-old moroccan woman who presented with a 2-year history of a swelling in her right preauricular region. Histological examination revealed the typical appearance of a nodular hidradenoma. The tumor was excised and one year after the initial presentation, there was no sign of recurrence. We emphasize the importance of wide surgical excision with appropriate margins to prevent local recurrence. A close follow up of the patients is recommended.

RESUMEN

INTRODUCTION: Lemierre's syndrome is a rare and potentially fatal entity characterized by the spread of an oropharyngeal infection, with secondary suppurative thrombophlebitis of the internal jugular vein and septic emboli. PRESENTATION OF CASE: We discuss the case of a 52-year-old male who developed Lemierre's syndrome following peritonsillar abscess. He presented with submandibular and submental swelling extending into the neck. His management included; incision and drainage of the abscesses; and prolonged anticoagulant therapy. CONCLUSION: The incidence of Lemierre's disease appears to be increasing, perhaps due to ignorance of the disease by many clinicians, and diagnosis is often delayed with potentially fatal consequences.

RESUMEN

INTRODUCTION: Malignant melanomas (MM) of the parotid gland are rather rare and when discovered in the parotid gland without an identifiable primary site, they are considered even rarer. PRESENTATION OF CASE: We report a case of a 27 years-old woman who was admitted to our department with a complaint of a painless mass in the parotid area. Clinical examination and radiological investigations found a mass compatible with pleomorphic adenoma. Treatment consisted of superficial parotidectomy. The evolution was marked by the appearance of a tumefaction regarding the scar. A total parotidectomy with neck dissection was performed and the histopathological examination found an intraglandular melanoma. CONCLUSION: Primary malignant melanoma may represent a serious diagnostic challenge. It should be considered in the differential diagnosis of parotid tumors even if it's rare.

RESUMEN

The pyriform sinus fistula is a rare condition described as an epithelialized tract connecting the skin of the neck to the foregut, and may result in cervical cysts and iterative abscesses misleading the diagnosis. The clinical and radiological examinations are all useful. Surgery stands as one of the most effective therapeutic options consisting on the total excision on the eventual cyst, and the fistula that is followed to its inner opening on the pyriform sinus. We present a case of a 3-years-old boy with a pyriform sinus fistula that caused recurrent neck abscesses treated independently delaying the diagnosis. Once in our structure, after radiological examination and antibiotics to cool the infection down, the surgery removed the cyst with its tract that opened in the pyriform sinus. The follow up showed an effective result with the total disappearance of the lesion with no more infectious episodes. Even if it's a rare condition, the diagnosis of apyriform sinus fistula must be considered in front of every patient with a history of recurrentlatero cervical abscess.

RESUMEN

INTRODUCTION: Hidradenoma papilliferum, also known as papillary hidradenoma, is a rare tumor of the sudoral glands, that occurs almost exclusively in women, on the vulvar and perineal region. Non-genital papillary hydradenoma is an even more rare occurrence, though some cases have been described in literature.This is a report of a 56 years old patient who presented with unilateral hearing loss secondary to a mass of the external auditory canal (EAC). A biopsy allowed us to make the diagnosis of a papillary hidradenoma of the EAC. The treatment was based on surgery; the tumor was removed while respecting the adjacent structures. Follow-up of the patient shows no actual recurrence. CONCLUSION: Ectopic Hidradenoma papilliferum located in the EAC is a rare occurrence, and should be considered as a possible diagnosis when investigating a mass of the EAC.

RESUMEN

INTRODUCTION: Bezold abscess is a suppurative complication of mastoiditis that the incidence has significantly decreased in the current era due to the introduction of antibiotics. PRESENTATION OF CASE: We discuss the case of a 62-year-old male who developed Bezold abscess following a right mastoiditis. He presented with laterocervical swelling. His management included; incision and drainage of the abscesses; and mastoidectomy. CONCLUSION: The incidence of Bezold abscess appears to be increasing, perhaps due to ignorance of the disease by many clinicians, and diagnosis is often delayed with potentially fatal consequences.

RESUMEN

INTRODUCTION: Rhabdomyosarcoma (RMS) is a malignant mesenchymal neoplasm uncommon in adults. PRESENTATION OF CASE: We report the case of an alveolar rhabdomyosarcoma in a rare location and in an adult. It concerns a 46-year-old woman with a mandibular location invading the parotid gland. The Immunohistochemical analysis revealed intense positivity for myogenin and desmin, favoring the diagnosis of alveolar rhabdomyosarcoma. The patient had a right hemi-mandibulectomy with parotidectomy and received chemotherapy with radiotherapy. The evolution was good up to 2 years postoperatively then the patient relapsed with a recurrence of tumour rapidly progressing and metastases in the cervical spine. DISCUSSION: RMS is an aggressive but rare disease that is one of the most common malignant head and neck tumors in children. The predilection sites of adult rhabdomyosarcoma are the extremities. Current treatment includes a combination of ablative surgery, chemotherapy, and radiation therapy. CONCLUSION: The prognosis of head and neck rhabdomyosarcomas in adults remains very poor given the often initially advanced stage of the disease and the high metastatic potential.

RESUMEN

Schwannomas are benign tumours arising from Schwann cells in the peripheral nerve. The schwannoma of the accessory nerve is a very rare entity. We report a case of Schwannoma of the extracranial accessory nerve. A 22-year-old man presented with a slow-growing mass, located on the right upper neck. The patient did not have any neurological deficit. CT scan showed a hypodense mass behind sternocleidomastoid muscle. The suspected diagnosis was an adenopathy of the accessory spinal chain. Surgery was done via transcervical approach. The histopathological analysis concluded with a diagnosis of schwannoma. No recurrence was noted at the follow-up examination 29 months after surgery.

Asunto(s)

RESUMEN

OBJECTIVES: To compare the outcome of patients with unilateral CRSsNP (U CRSsNP) and bilateral CRSsNP (B CRSsNP) undergoing FESS. Also, we evaluate the impact of SNOT-22 domains to predict their quality of life (QOL) outcomes and compare these factors with those of CRSwNP group, published in previous work. METHODS: A prospective cohort study was performed in the hospital 20 August,66 patients who were presented between January 2016 and December 2017 were diagnosed with CRS according to guideline recommendations, and were beforehand refractory to initial medical therapy and elected to FESS. The Sino Nasal Outcome Test-22 (SNOT-22) was used to evaluate QOL. RESULTS: A higher significant improvement was observed between preoperative and postoperative SNOT-22 scores in U CRSsNP group [37.13 ±â€¯9.307 versus 14.11 ±â€¯8.531] and in B CRSsNP group [41.76 ±â€¯6.949 versus 18.57 ±â€¯8.495]. In the U CRSsNP group, patients having a preoperative SNOT-22 score higher than 20 points attained MCID in 88%. For the other group, patients having preoperative SNOT-22 score superior to 40 points achieved MCID in 66%. A multivariate logistic regression model found preoperative predictors that have impact on QOL outcomes. CONCLUSIONS: Outcomes from this study suggest that patients with U CRSsNP having a preoperative SNOT-22 scores between 10 and 19, and patients with B CRSsNP having a preoperative SNOT-22 scores between 10 and 19 or 20-29 had no chance of achieving an MCID improvement after FESS. Also, preoperative rhinologic symptoms and preoperative psychological dysfunction domains of SNOT-22 are helpful tools to predict improvement after FESS unlike the unilateral character of CRS.