Myxoid Liposarcoma of the Parotid Gland.

Liposarcoma is extremely rare in the parotid gland. In this article, we report the case of an 87-year-old man who presented to our department with swelling in the left parotid region. Magnetic resonance imaging (MRI) revealed a tumor in the superficial lobe of the parotid gland. Total parotidectomy and ipsilateral lymph node dissection were performed. Histologic examination confirmed the diagnosis of myxoid liposarcoma of the parotid gland. On imaging, there was no evidence of nodal or distant metastasis. Radiation therapy was planned, but the patient refused to receive treatment. After a follow-up of 3 years, MRI showed no sign of tumor recurrence and the patient remained symptom-free. The case is original by the tumor's location. Only 8 cases of primary parotid liposarcoma have been reported in the literature. The purpose of this article was to discuss, through our case, clinical and anatomopathological features of parotid gland liposarcomas as well as its treatment options and prognosis.

Undifferentiated Small Round Cell Sarcoma of the Hypopharynx in a 7-Month-Old Patient.

Undifferentiated small round cell sarcoma is an extremely rare and highly aggressive tumor touching the soft tissues and bones. Here, we report the case of a 7-month-old girl who presented to our department with respiratory distress due to a hypopharyngeal mass causing airway obstruction. The patient underwent a tracheotomy and had a direct laryngoscopy. Histological examination confirmed the diagnosis of an undifferentiated small round cell sarcoma. The patient received chemotherapy and radiotherapy and underwent neck dissection. One year later, the patient was diagnosed with local recurrence as well as metastatic cervical nodes and liver and pulmonary metastases. The case is original by the tumor's location and the age of onset. To the best of our knowledge, this could be the first case in English and French literature reporting an undifferentiated small round cell sarcoma affecting the hypopharynx among infants. The purpose of this article is to report our case and discuss its clinical and anatomopathological features as well as its prognosis and treatment options.

Clinical Features of Systemic Lupus Erythematosus in Tunisian Males.

PURPOSE: Systemic Lupus Erythematosus (SLE) in males is rare. Clinical and biological features, as well as, the outcome may differ comparatively to female patients. The purpose of our study is to define these clinical and biological features in Tunisian male patients presenting SLE. METHODS: A mono-centric, retrospective and descriptive study of 96 patients followed for SLE out of which 21 are males. A comparative study was then performed between male and female patients groups. RESULTS: Sex-ratio female/male was 3.6/1, the average age at diagnosis of SLE was 37.8±14 years. The most frequently noted clinical manifestations were: skin involvement (81%), renal involvement (71.4%) and joint damage (66.7). We observed a significant difference in clinical features between male and female patients (21 males and 76 females): renal failure (52% vs. 71.4%), serositis (23.8% vs. 2.7%), peripheral neuropathy (19% vs. 4%) and lung interstitial disease (14.3% vs. 1.3%). No significant difference was found in the positivity of serum antibodies between the two groups. Fifteen male patients (71.4%) had a SLEDAI score greater than or equal to 11, referring to high/very high disease activity. Out of the 32 patients who developed infectious complications during the course of the disease, 11 were male (52.4% of males). Concerning the male group, complete remission was observed in 10 patients (47.6%), while 10 others presented persistent sequella. We observed one death in the male group secondary to infective acute respiratory failure. CONCLUSION: SLE in male patients is rare and associated with poor prognosis. Disparity was observed in clinical and biological features as well as outcome in the different studies. In our study, we concluded that male lupus is more severe.

[Dehiscence of the lamina papyracea]. / Déhiscence de la lame papyracée.

We report the case of a 35 year old patient presenting with acute onset of right periocular pain with palpebral edema due to nose blowing with absence of trauma. Early assessment showed periorbital emphysema without ophthalmological involvement, normal pupillary light reflex and visual acuity. Computerized tomography (CT) scan of facial and orbital bones objectified right pneumo-orbita with orbital fat herniation via a partial agenesis of the lamina papyracea, without muscle incarceration. Emphysema gradually reduced after two weeks by applying pressure dressing associated with broad spectrum antibiotic therapy as well as following the rules for cleanliness (avoiding closed glottis efforts, nose blowing…). Pneumo-orbita is defined by the presence of orbital air. It is mainly caused by a trauma but few cases of spontaneous dehiscence have been reported. The opening of the lamina papyracea, acting as an antireflux valve, prevents air outlet. CT scan is essential to confirm the diagnosis. It most often has a spontaneous resolution but the presence of hyperpression, that can be responsible for ischemic optic neuropathy or occlusion of the central artery of the retina, require a rigorous monitoring in order to implement possible urgent decompressive intervention.