Pathologic features of head and neck adult rhabdomyosarcoma: Case series.

INTRODUCTION: Adult Head and neck Rhabdomyosarcomas (HNRMS) are exceedingly rare and remain challenging for pathologists. CASES PRESENTATION: Five cases of adult HNRMS (≥19 years) were retrieved from the archives of the Department of Pathology of Hospital of Specialities in Rabat (HSR) in Morocco, over 5 years. Clinical and pathologic findings from hematoxylin and eosin slides and immunohistochemistry for Desmin and Myogenin were reviewed. CLINICAL DISCUSSION: The median age was 33, with a men's predominance (3 M/2F). Histological analysis revealed three cases of Alveolar Rhabdomyosarcoma (RMS), one Pleomorphic RMS, and one spindle cell/sclerosing RMS. In addition to the typical histology observed in each RMS, we found tricky growth patterns that could be a source of misdiagnosis. All five cases demonstrated variable positivity for Desmin and Myogenin. CONCLUSION: HNRMS cases have different pathological features than pediatric RMS cases. We identified rare subtypes such as pleomorphic and spindle cell/sclerotic RMS, which exhibit unusual morphological patterns.

An unusual location of a cavernous hemangioma: a case report.

Hemangiomas are benign vascular tumors that most often affect the skin, mucous membranes, subcutaneous tissues, bone and on rare occasions muscles. In the head and neck region, the masseter and trapezius muscles are most often affected; the temporalis muscle involvement is extremely rare. It is a childhood pathology that rarely occurs in adults. We report a case of a cavernous hemangioma in a 37-year-old female. Through this case and in the light of literature we focus on the clinicopathological aspects of this tumor and the rarity of this location.

[Median mandibular cleft in adults: a case report and literature review]. / Fente mandibulaire médiane chez l'adulte: à propos d'un cas et revue de la littérature.

Orofacial clefts are common congenital malformations. Tessier's classification system is the most widely used; it consists of 30 variants including median mandibular clefts (Tessier's cleft No. 30). They can be isolated or associated with cleft of the lower lip, tongue or more rarely, of the sternum. They are very rare, less than 70 cases (all types taken together) have been reported in the literature. We here report an exceptional case of mandibular and sternal cleft with literature review.

[Therapeutic management and prognostic profile of a patient with maxillary sarcomatoid carcinoma (case report)]. / Carcinome sarcomatoïde: prise en charge thérapeutique et profil pronostique à propos d´une localisation maxillaire inhabituelle (à propos d´un cas).

Sarcomatoid carcinoma is a rare, aggressive, malignant tumor with a poor prognosis and a very high frequency of recurrence. Carcinoma of the maxillary sinus is extremely rare. We report the case of a 42-year-old woman with left maxillary process. Biopsy revealed aggressive sarcomatoid carcinoma with a lymph-node metastasis. The patient underwent surgical excision, lymph-node dissection followed by radiotherapy with good outcome. The rarity of this site-specific cancer poses a problem of diagnosis and timely management which is still a controversial topic. However, wide surgical excision is the gold standard treatment. This study highlights the anatomoclinical peculiarities and, in particular, the prognostic features of this tumor.

Solitary fibrous tumor occurring in the parotid gland: a case report.

BACKGROUND: Solitary fibrous tumor is an uncommon spindle cell neoplasm of unknown origin. It has been reported in many anatomic sites, with a rare occurrence in the head and neck region. Solitary fibrous tumors of the parotid gland are exceptional; their clinical and radiologic features are non specific, often mimicking more common salivary gland tumors. Pathologic examination and immunohistochemistry are required to make the correct diagnosis. The prognosis is favorable, with most tumors being benign, and complete surgical resection is the treatment of choice. CASE PRESENTATION: We report the case of a 42-year-old man who presented with a painless mass involving the parotid gland. A parotidectomy was performed, and follow up was unremarkable. Gross examination showed a well circumscribed, firm tumor measuring 3,4 cm. Histologically, the tumor was composed of a spindle cell proliferation of variable cellularity, with staghorn vessels. A panel of immunohistochemical stains was performed, and confirmed the diagnosis of parotid gland solitary fibrous tumor. CONCLUSION: In this report we aim to increase awareness of this rare entity among clinicians and pathologists, and to emphasize the role of immunohistochemistry in confirming the diagnosis.

Giant pediatric cervicofacial lymphatic malformations.

PURPOSE: Lymphatic malformations (LMs) are benign lesions. Most of them are found in head and neck regions as asymptomatic mass, but giant lymphangiomas may affect breathing or swallowing and constitute a major therapeutic challenge. METHODS: A retrospective analysis of giant head and neck LMs with impairment of respiration or swallow for the past 11 years was performed in the Department of Maxillofacial Surgery and ENT of the Avicenne Medical University Center. RESULTS: Seven patients with large and extensive LMs of the head and neck were identified. There were 3 males and 4 females with a mean age of 6 years. The predominant reason for referral was airway compromise necessitating tracheostomy (57%) and dysphagia (43%). Three patients had macrocystic lesions; others were considered mixed or microcystic. All the patients underwent surgical excision as a primary treatment modality. Complete surgical resection was realized in 4 patients, and subtotal resection in 3 patients. Of 7 patients, 4 patients had complications including nerve damage and recurrence of the disease. The majority of the patients underwent only a single surgical procedure. CONCLUSIONS: Cervicofacial LMs in children should be managed in multidisciplinary setting. Surgery remains the first treatment for managing giant, life-threatening lesions.

Primary extranodal non-Hodgkin lymphoma of the oral cavity.

Lymphoma is the second most common neoplasm of the head and neck after squamous cell carcinoma. Nearly 24% to 48% of non-Hodgkin lymphoma can arise in extranodal localizations and 3% to 5% of which were primarily located in the oral cavity. Waldeyer ring is the most frequently involved site. The authors report 2 cases of primary extranodal non-Hodgkin lymphoma of the oral cavity, not of the Waldeyer ring.

Primary oral malignant melanoma.

Malignant melanoma of the oral cavity is a rare neoplasm. It is well known for his poor prognosis and the need for an evidence-based treatment. Therapy is commonly based on surgical excision of the primary tumour, supplemented by adjuvant therapy. In this paper two new cases of primary oral melanoma are reported with a review of the literature.

Mucoepidermoid carcinoma arising from pleomorphic adenoma of the soft palate.

Carcinoma arising in pleomorphic adenoma is a rare entity. A case of mucoepidermoid carcinoma in pleomorphic adenoma occurring in the soft palate of a 40-year-old woman is reported. An intraoral excision of the tumor was performed. Histopathological examination revealed high-grade mucoepidermoid carcinoma proliferated in pleomorphic adenoma with free surgical margin. The patient received adjuvant neck radiotherapy. She remains free of disease 16 months post-treatment.