[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.

[Cutaneous blastomycosis revealing intravascular B-cell lymphoma: a case in Morocco]. / Blastomycose cutanée révélant un lymphome B intravasculaire: une observation marocaine.

Blastomycosis is a systemic fungal infection caused by a thermally dimorphic fungus, Blastomyces dermatitidis. The incidence in immunocompromised patients has increased in the last two decades. A 55-year-old man consulted for inflammatory nodules on the forearm. Biopsy of one nodules showed a pseudoepitheliomatous hyperplastic epidermis overlaying a dense agranulomatous inflammatory infiltrate containing free-formed ovoid bodies enclosing giant macrophageous cells. These findings were consistent with blastomycosis. After a month of treatment cutaneous lesions regressed partially but the patient's general status continued to deteriorate with the appearance of an edematous-ascitic syndrome and icterus. Laboratory blood testing demonstrated cholestasia and abdominal ultrasound showed hepatosplenomegaly. Needle liver biopsy revealed giant B-cell lymphomatous infiltration of the hepatic ducts. The patient's condition worsened rapidly and he died five months after diagnosis despite four rounds of chemotherapy. Blastomycosis is rare in Morocco. Primary infection is usually a pneumonic process. Isolated cutaneous infection is possible but uncommon. To our knowledge the association of blastomycosis and intravascular lymphoma has not been previously reported. In immunocompromised patients, clinical findings can be alarming and the outcome can be rapidly fatal.

[Angiolymphoid hyperplasia with eosinophilia]. / Hyperplasie angiolymphoïde à éosinophiles.

INTRODUCTION: Angiolymphoid hyperplasia with eosinophilia is a rare affection. CASE REPORT: A 36 years-old woman presented an angiolymphoid hyperplasia with eosinophilia manifesting by erythematous nodular lesions on the face and the forearms, severe neuropathy, visual loss and eosinophilia. Corticosteroids, immunosuppressive drugs and interferon-alpha were unsuccessful. DISCUSSION: Angiolymphoid hyperplasia with eosinophilia is a dermatological disease with possible systemic features. Differential diagnosis with Kimura disease is based on clinical presentation and histological findings. Etiopathology remains unknown and the treatment is not codified.

[Spheno-orbital mesenchymal chondrosarcoma. A case report]. / Chondrosarcome mésenchymateux sphéno-orbitaire. A propos d'un cas.

Mesenchymal chondrosarcoma is a highly malignant and extremely rare tumor of the orbit: only 18 cases have been reported to date. We report a case of spheno-orbital mesenchymal chondrosarcoma in a 36-year-old woman presented with a 4-month history of progressive left exophthalmia and temporal mass. A CT-scan of the orbit and MRI showed a spheno-orbital mass, with temporal fossa extension, fed by the internal maxillary artery visible on cerebral angiography. Surgery via a transcranial, left frontotemporozygomatic approach after selective embolization enabled subtotal removal. Definitive histologic examination revealed mesenchymal chondrosarcoma. Postoperatively, exophthalmia spectacularly regressed. We report our clinical findings and present a review of the literature.

[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.

[Small cell neuroendocrine carcinoma of the bladder. A new case report]. / Tumeur neuro-endocrine à petites cellules de la vessie. Une nouvelle observation.

This rare but aggressive bladder tumour presents the morphological and immunohistochemical characteristic common to all neuro-endocrine tumours observed in other organs. The authors report a case of primarymalignant neuro-endocrine bladder tumour. Combined surgery-cisplatin-based adjuvant chematherapy is recommended.

[Benign granular cell anal tumor]. / Tumeur anale bénigne à cellules granuleuses.

INTRODUCTION: Granular cell tumors (GCT) are rarely located in the perianal area. OBSERVATION: Over the past 3 years, a 56 year-old man presented a papule of the right margin of the anus that had progressively increased in size (1.5 cm). Cell proliferation was located in the dermis and strongly expressed the S100 protein. It was covered by a pseudo-epitheliomatous hyperplasia of the overlying epidermis. Forty months after local surgical excision, there was no sign of recurrence. COMMENTS: Granular cell tumors are rare and usually benign. When cutaneous or mucosal, the pseudo-epitheliomatous hyperplasia of the overlying epithelium may, on superficial samples, be mistakenly diagnosed as squamous cell carcinomas. Malignant GCT may, histologically, appear identical to a benign GCT and only the appearance of metastases (generally after local recurrence) permits the subsequent diagnosis of malignancy.

[Botryoide sarcoma of the uterine cervix. Apropos of 1 case]. / Sarcome botryoïde du col utérin. A propos d'un cas.

Sarcoma botryoide (SB), a variant of rhabdomyosarcoma, is a rare tumor who are rarely localized in the uterine cervix. Only 99 cases have been described to date. The authors reported this case with review of the literature in order to insist on diagnosis progress and a new combined modality approach using: radiotherapy, surgery and chemotherapy which have improved prognosis in the localized disease especially.

[Cutaneous blastomycosis revealing a corticoadenoma]. / Blastomycose cutanée révélant un corticosurrénalome.

BACKGROUND: Blastomycosis is an uncommon chronic granulomatosis caused by Blastomyces dermatitidis. We present a case with a skin localization that disclosed malignant corticoadenoma. CASE REPORT: A 32-year-old man consulted for inflammatory nodules of the face and lower limbs. The histological examination of a nodule biopsy was in favour of blastomycosis. Ketoconazole treatment was ineffective. Amphotericin B provided cure of the skin lesions. Search for extension disclosed a malignant corticoadrenoma. DISCUSSION: Blastomycosis is very rare in Morocco. The portal is usually the lung. Unique skin involvement is very exceptional. To our knowledge this is the first report of a blastomycosis-corticoadenoma association. The clinical situation may be alarming in immunodepressed subjects. Amphotericin B treatment is indicated.

[Nodular fasciitis or pseudosarcoma]. / Fasciite nodulaire ou pseudosarcomateuse.

Nodular fasciitis is a benign neoplastic and reactive proliferation of fibroblasts of soft tissues, which is often mistaken for a sarcoma because of its rapid growth, rich cellularity and mitotic activity. A case is reported that provides the opportunity to discuss the specific clinical and pathological features of nodular fasciitis.

[Small cell carcinoma of the prostate. Apropos of a case]. / Le carcinome à petites cellules de la prostate. A propos d'un cas.

Small cell carcinomas of the prostate are rare. These tumors exhibit morphologic and functional neuro-endocrine characteristics. They are a highly aggressive. New therapeutic protocols are being developed in order to be effective against these tumors, particularly those unresponsive to hormonotherapy.