[Infratemporal metastasis from a renal cell carcinoma]. / Métastase infratemporale d'un carcinome à cellules rénales.

INTRODUCTION: Kidney cancer, particularly clear cell carcinoma, has an unpredictable clinical course with a variable metastatic potential in time. Some metastatic sites are atypical, such as head and neck (6% of cases). We report an extremely rare case of clear cell renal carcinoma with a metastasis in the infratemporal fossa. OBSERVATION: A 67 year old male patient, operated in 2007 for a Fuhrman grade I clear cell carcinoma of the left kidney (radical nephrectomy), consulted for swelling of the left temporo-zygomatic region. A local biopsy allowed diagnosing clear cell renal carcinoma metastases. The patient was inoperable and was treated by antiangiogenic drugs. DISCUSSION: The recommended treatment is surgical exeresis when the metastasis is single and can be resected. Antiangiogenic therapy is an alternative in other cases.

[Metrorrhagia disclosing a synchronous bilateral breast cancer: report of a case]. / Métrorragie révélatrice d'un cancer du sein bilatéral synchrone: à propos d'un cas.

Genital metastases are very rarely indicative of breast cancer; they are exceptionally located at the cervix. These atypical locations are more common when it comes to a metastatic breast cancer or a histological infiltrating lobular type. The simultaneous association of a lobular and a ductal infiltrating cancer under a synchronous bilateral breast cancer still remains a rare entity. In this work, we report the observation of a woman aged 48 who has a synchronous bilateral breast cancer, of different histological types, and who reported at first a genital bleeding which is caused by a metastasis in the cervix of the uterus.

[Rosai-Dorfman disease mimicking meningiomatosis: a case report]. / Maladie de Rosai-Dorfman mimant une méningiomatose: à propos d'un cas.

Rosai-Dorfman disease (RDD), also known as sinus histocytosis with massive lymphadenopathy, is a rare idiopathic histioproliferative disease affecting the lymph nodes. Although extranodal involvement has been reported in diverse sites, central nervous system manifestations, particularly in the absence of nodal disease with clinical and radiological findings suggestive of meningioma, are extremely rare. Histopathology and immunohistochemistry are essential for a positive diagnosis. We report a case of RDD in a patient presenting multiple meningeal nodules with a review of the literature and discussion of differential diagnosis.

[Erysipelas of the face revealing a mucormycosis]. / Érysipèle de la face révélant une mucormycose.

BACKGROUND: Mucormycosis is an opportunistic fungal infection, typically affecting immunocompromised patients. Rhino-orbital location is the most frequent form with cerebral blood vessels invasion and a fatal outcome. CASE REPORT: An immunocompetent 38-year-old woman, with previous history of primary cutaneous mucormycosis, was admitted for a febrile erythemato-oedematous lesion of the face with well-demarcated edge evoking erysipelas. No cutaneous portal of entry was identified. Oral cavity examination found an ulceronecrotic lesion of the hard palate. Diagnosis of mucormycosis was retained after nasal endoscopy and histological findings. The patient was first treated for erysipelas, then by ascending dose of intravenous amphotericin B. A good outcome was observed despite the occurrence of nephrotoxicity which normalized by alkaline hyperhydratation. Healing was obtained after 1month of effective dose leaving cleft palate sequelae. DISCUSSION: Mucormycosis occurs rarely in immunocompetent, even more if it is a second episode. Affecting the face, it may simulate or be revealed by superficial skin infection. Within that, a meticulous otorhinolaryngeal examination is required when the portal of entry is not obvious.

Acral myxoinflammatory fibroblastic sarcoma.

Acral myxoinflammatory fibroblastic sarcoma is a rare low-grade malignant soft tissue tumor, usually observed in the extremities of middle-aged patients. We report a case involving the third finger of the left hand of a middle-aged man. The tumor showed a nodular architecture, with cellular areas, occasional foci of hyalinized fibrosis, and hypocellular areas with a myxoid background. Various neoplastic cells were identified including spindled or rounded epithelioid cells and occasional bizarre giant cells, morphologically mimicking ganglion cells. Tumor cells were strongly immunoreactive for vimentin and variably positive for CD68 and CD34. The tumor was completely removed, without further treatment.

[RACAND syndrome associated with primary biliary cirrhosis]. / Syndrome RACAND associé à une cirrhose biliaire primitive.

The acronym RACAND means the association of Raynaud's phenomenon, anticentromere antibodies and digital necrosis without digital sclerosis. It is a rare syndrome recently individualised. The association with primary biliary cirrhosis has never been previously reported, and leads to discuss its nosology. A 57-year-old woman with a history of Raynaud's phenomenon, presented with recurrent episodes of fingers and toes necrosis. Clinical examination did not evidence digital sclerosis. Anticentromere antibody titer was high. There was no oesophageal or lung involvement. A liver biopsy performed because of moderate increase in liver enzymes showed histological lesions of primary biliary cirrhosis. Treatment with iloprost, platelet aggregation inhibitors and anticalcic drugs could not avoid amputation of several toes. It is possible that anticentromere antibodies are directly toxic to vascular endothelial cells and result in a diffuse or localized vasculopathy. The association with primary biliary cirrhosis is in favour of autoimmune condition of both vascular and ductular endothelial cells.

[Nasoethmoidal psammomatoid cemento-ossifiying fibroma with intraorbital extension]. / Fibrome cémento-ossifiant psammomateux nasoéthmoïdal à extension orbitaire.

INTRODUCTION: Psammomatoid cemento-ossifying fibroma (PCOF) is a rare benign fibro-osseous lesion. It affects the maxilla, the nasal cavity, paranasal sinuses, and the ethmoid. It is slow-growing, progressive, and benign but it can be locally extended and mimic a malignant tumor. OBSERVATION: We report the unusual case of a 36-year-old woman with a nasal and ethmoid PCOF with orbital and endocranial extension. The tumor was revealed by exophthalmia and nasal obstruction. The diagnosis was proven histologicaly on biopsies. The treatment was transfacial surgery. There was no recurrence 1 year later. DISCUSSION: Intraorbital and endocranial extension of PCOF are rare aggressive behaviours. Histologically, the differential diagnosis is difficult between fibrous dysplasia or psammomatoid meningioma. Complete surgical excision is necessary to prevent recurrence.

[An historical case of malignant hyperparathyroidism with unusual metastatic sites]. / A propos d'une hyperparathyroïdie maligne historique à localisations métastatiques inhabituelles.

We report a historical case of hyperparathyroidism in a young patient hospitalized for an array of osteolytic foci and incomplete fracture associated with a swollen neck, revealing a very special form of a metastatic parathyroid carcinoma with unusual multiple locations and exceptional medullary flooding. Carcinoma of the parathyroid gland produces a malignant hypersecreting tumor particularly difficult to diagnose. Treatment of this rare tumor is primarily surgical. The preoperative syndrome is unusually severe primary hyperparathyroidism. Intraoperatively, the size of the tumor and its local extension to surrounding tissue are highly suggestive. Confirmation requires pathological analysis of the operative specimens and can be further supported by the clinical course of local recurrence or metastasic spread. Specific immunohistochemical techniques have recently been shown to be contributive. The diagnosis is strengthened in the presence of associated Schantz and Castelman criteria. Foci of local extension can be identified preoperatively with ultrasound, (99m)Tc-sestamibi scintigraphy and MRI of the neck and mediastinum. The prognosis depends mainly on the possibility of achieving complete resection at the initial surgery. In some cases, very aggressive complementary postoperative radiotherapy is likely to improve locoregional control of the tumor. Chemotherapy alone or in combination with radiation has not demonstrated its effectiveness. The disease course and control can be monitored by regular assay of serum calcium and the parathormone.

[Desmoplastic ameloblastoma in a 7 year old child]. / Améoblastome desmoplastique chez un enfant de 7 ans.

Desmoplastic ameloblastoma is a benign, locally aggressive neoplasm of proliferating odontogenic epithelial origin. It is seen among old patients from 17 to 72 years with an average age 42 years and without predilection of sex. We report the case of a 7 year old child, having presented since the 5 years age, a gingival tumefaction on the left higher incisivo-canin group which increased volume gradually. The stomatologic examination showed a gingival tumefaction covered with a healthy mucous membrane, ovoid form and measuring 3 cm on its horizontal axis. The tomodensitometry of the jawbone showed in front of the 21st and the 22nd tooth, the presence of an osseous lesion associating of the hearths of osteolysis and osteocondensation with rupture of cortical and invasion of the soft tissue. A curetting of the lesion was carried out and the anatomopathologic examination retained the diagnosis of desmoplastic ameloblastoma. The characteristic of our observation is the youth of the patient. In addition, the desmoplastic ameloblastoma is relatively rare, is characterized by an anatomical distribution, a radiological appearance and a morphological aspect differents from the traditional ameloblastoma. A radical surgical treatment is suggested for this tumour to avoid recurrency.

[Ganglioneuroma of the zygoma]. / Le ganglioneurome de l'os zygomatique.

INTRODUCTION: Ganglioneuroma is an uncommon benign tumor that arises from the sympathetic nervous system accounting for less than 1% of all soft-tissue neoplasms. CASE REPORT: We report the case of a 22 year-old man who presented a ganglioneuroma localized in the zygoma. DISCUSSION: Ganglioneuroma generally develop in the abdomen or thorax soft tissue. The occurrence of ganglioneuroma in the bone is exceptional. To date less than ten cases have been reported, six of which involved the mandible. We discuss the possible causes of tumor development at this site.