An Uncommon Presentation of Adrenal Cyst with Subclinical Cushing's Syndrome: A Diagnosis Dilemma.

Adrenal cysts are a rare entity that is usually nonfunctional and asymptomatic. Their association with adrenal neoplasms was rarely described in the literature. We report a unique case of a 40 -year-old woman who was referred for evaluation of a left adrenal incidentaloma with subclinical Cushing's syndrome. The tumor was suspicious for malignancy regarding computed tomography scan (CT scan) features. Therefore, a laparoscopic left partial adrenalectomy was performed. Pathology examination showed multilocular spaces lined by endothelial cells which are compatible with endothelial adrenal cyst, associated to an adrenocortical adenoma. We further discuss the management of adrenal cyst with review of the literature.

[Wells syndrome misdiagnosed as bacterial cellulitis]. / Syndrome de Wells mimant une cellulite bactérienne : un piège diagnostique et thérapeutique.

Wells syndrome (WS), also called eosinophilic cellulitis is a rare inflammatory skin disease with about 200 cases reports in the literature. It is part of eosinophilic dermatoses and presents a diagnostic challenge. We report a case of WS presenting as bacterial cellulitis. A 59 year-old woman was referred to our department with a 10-day history of a rapidly extending erysipelatoid rash of the left upper limb, non-responding to oral antibiotics. Upon physical examination, erythema with swelling and blisters were found on the left upper limb with urticarial-like lesions on the breast, the belly and on the inner thighs. The patient was admitted with presumed bacterial cellulitis and was treated with intravenous ampicillin. Blood count showed raised eosinophils (2050/ul) along with a biological inflammatory syndrome. Biopsies were performed on both bullous and urticarial-like lesions showing perivascular and interstitial inflammatory infiltrate, made essentially of eosinophils with flame figures. The diagnosis of WS was made and the patient received local and oral corticosteroids (0,5mg/kg/d) with good evolution. The rarity and clinical manifestations of WS makes it sometimes difficult to differentiate between a WS and a bacterial cellulitis. However, biological and histological findings can help the clinician make the differential diagnosis between these two diseases for a better management.

[Giant seborrhoeic keratosis in an unusual site]. / Kératoses séborrhéiques géantes de siège inhabituel.

BACKGROUND: Seborrhoeic keratoses (SK) are benign tumours preferentially located on the head and trunk. We report a case of giant SK at an unusual site. PATIENTS AND METHODS: An 85-year-old man consulted for pigmented genital lesions present for 10 years. Clinical examination revealed small pigmented formations evoking SK and other pigmented, confluent and hyperkeratotic bordering plaques with a circinate contour. The latter lesions were biopsied, and histological findings were characteristic of SK and showed epidermal acanthosis associated with orthokeratotic invagination of the mucosa, resulting in the formation of characteristic wells. Treatment by electrocoagulation curettage was undertaken. DISCUSSION: SK are benign tumours that occur primarily in patients aged over 50 years. They are preferentially located in the trunk, head and neck. A genital site of SK is very unusual, with no more than 10 published cases. The cause of genital SK is as yet unknown, but data in the literature point to a possible role of chronic friction and HPV.

Aggressive ovarian psammocarcinoma: a case report.

Serous psammocarcinoma is a rare form of ovarian carcinoma characterized by massive psammoma body formation. We report a new case of aggressive ovarian psammocarcinoma with omental and peritoneal implants.

[Baker Rosenbach erysipeloid appearing as a granulomatous cheilitis]. / Erysipéloïde de Baker-Rosenbach prenant l'aspect d'une chéilite granulomateuse.

BACKGROUND: Baker-Rosenbach's erysipeloid is a skin infection caused by Erysipelothrix rhusiopathiae. It occurs essentially in humans exposed to animals colonized with this germ such as swine. The typical skin lesion, an erythematous macule generally localized to inoculation site, frequently in the extremities, quickly resolves spontaneously. The lips are an atypical site of this infection. We describe a case of chronic granulomatosis cheilitis in a farmer caused by E. rhusiopathiae. CASE REPORT: A 40-year-old farmer, a wild-boar hunter and chronic smoker with no history of tuberculosis, injury or insect bites, presented at our dermatology unit with ulcerative macrocheilitis of the lower lip ongoing for 1 year. Its surface was purulent. A biopsy specimen showed non-caseating epithelioid granulomas. Laboratory and radiological screening for tuberculosis, sarcoidosis and Crohn's disease, and parasitological examination for Leishmaniasis proved negative. Bacteriological examination identified E. rhusiopathiae and labial Baker-Rosenbach's erysipeloid was diagnosed. The lesion healed after 15 days of treatment with parenteral penicillin G (12m IU/d), totally disappearing after 3 months. DISCUSSION: Swine erysipelas usually occurs in man as Baker-Rosenbach's erysipeloid. This localized form of infection with E. rhusiopathiae is the most frequent and the lesion typically observed is a violaceous plaque, less inflammatory with induration; spontaneous regression occurs after a mean 3 months. To our knowledge, this case is the first report of ulceration associated with macrocheilitis. Histologically, the granuloma directed our investigation towards the principal aetiologies of granulomatosis cheilitis, such as tuberculosis considering the epidemiological context, sarcoidosis or Crohn's disease. The diagnosis of erysipeloid was supported by epidemiological evidence (occupational exposure), isolation of the germ at the lesion and its regression on treatment with penicillin G. CONCLUSION: Diagnosis of E. rhusiopathiae infection was confirmed by bacteriology. However, the hypothesis concerning the pathogenesis of its chronic course in our patient remains a subject of discussion.

[Solitary fibrous tumor of the scalp: a case report]. / Tumeur fibreuse solitaire du cuir chevelu : a propos d'un cas.

The solitary fibrous tumor is a rare tumor, ubiquitous, mesenchymatous essentially affecting serosal surfaces. The location in the scalp is exceptional. We report a case of tumor in left occipital scalp discovered after recurrence of a tumor initially taken for a lipoma. The motive for consultation was mainly aesthetic. The pathological examination is the main element of diagnosis. The assumption is based on a large lumpectomy and an extended and regular clinical monitoring. The forecast is favourable with a decline of one year. However, relapses may occur in the long-term and there are malignant forms.

[Oral verrucous carcinoma and human papillomavirus infection]. / Carcinome verruqueux buccal et infection à papillomavirus.

BACKGROUND: Verrucous carcinoma of the oral cavity is a clinical variant of squamous cell carcinoma. Infection with human papillomavirus (HPV) seems to be a significant risk factor in carcinogenesis, as illustrated in our case report. PATIENTS AND METHODS: A 72 year-old woman with a history of actinic cheilitis consulted for a bulky tumour of the lips and palate. Clinical examination revealed a highly infiltrated labial tumour vegetating and budding, with a thick edge. A bulky tumour and firm masses were seen on the hard and soft palates. Biopsy samples from both sites indicated well-differentiated veruccous epidermoid carcinoma with chorionic infiltration. The immunohistochemical study showed intestinal tumour containing HPV-16 virus. The central facial scan showed involvement of the nasal fossae, soft palate and lips with lysis of the upper maxilla arcade and the osseous palate. The patient died a few days before the start of preoperative chemotherapy following severe deterioration of her general state. DISCUSSION: Verrucous carcinoma is an authentic well-differentiated low-grade cancer. It appears as a wart-like exophytic lesion and progresses over several years. Diagnosis is based on histological examination. Management and treatment are not codified but surgery remains the treatment of choice and relapse is common in the case of locoregional involvement.

[Bullous lupus. About one case]. / Lupus bulleux. A propos d'un cas.

Bullous lupus is a rare manifestation of systemic lupus characterized by subepidermal blistering. We report a case of a-27-year-old woman with vesiculobullous lesions and class IV lupus nephritis. Bullous lupus is often associated with autoimmunity to type VII collagen.

[Liposarcoma of the thumb]. / Liposarcome du pouce.

Liposarcoma is the most common malignant deep soft-tissue tumor in adults. however, primitive cutaneous liposarcoma is very rare. We report the observation of a patient who presents a liposarcoma of the thumb.

[Primary hepatic localization of the PPNET (primitive peripheral neuroectodermal tumors). Case report]. / Les tumeurs neuroectodermiques primitives périphériques PPNET à localisation hépatique primaire. A propos d'un cas.

Our purpose is to study, through the case of a patient operated for right hepatic tumour, the clinical, radiological, anatomopathologic and therapeutic aspects of the peripheral primitive neuroectodermal tumours (PPNET). This tumour (PPNET) is a neoplasm belonging to the Ewing's family tumours, whose histology is similar. Its diagnosis requires the contribution of histopathology, immunohistochimy and cytogenetic studies. The primary hepatic localization of this rare tumour (our case), has never been reported. The treatment, in theory copied on that of the Ewing's sarcoma, is complex and not yet codified, which makes it another disappointing aspect of this disease whose prognosis remains dark.

[Management of phyllodes tumors of the breast at the National institute of oncology of Rabat, Morocco]. / Prise en charge des tumeurs phyllodes du sein à l'Institut national d'oncologie de Rabat, Maroc.

OBJECTIVES: Phyllodes tumors of the breast (PTB) are rare fibro epithelial tumors. Their terminology, histological classification and their treatment are exposed to controversy. The aim of our work is to underline the epidemiological, clinical, histological, therapeutical, prognostic and evolutive features of these tumours through a retrospective study and a review of the literature. PATIENTS AND METHODS: We reviewed at the National Institute of Oncology of Rabat, Morocco, between 1985 and 1998, all the files of patients with histological certainty of PTB, doubtful PTB were excluded. We collected epidemiological, clinical, histological, therapeutical, prognostic and evolutive features of these tumours. STATISTICS: quantitative parameters were represented by mean +/- S.D. and qualitative parameters by percentage or effective. RESULTS: We studied nine cases of PTB, which represented 0.09% of all primitive tumors of the breast treated at our institution during the study period. All our patients were female. Mean age was 37.3 +/- 10.07 years. Two of our patients (22.2%) had a history of fibroadenoma and 44.4% were nulliparous. Mean delay before consulting was 60.7 +/- 17.56 months and the median tumour size was 13 +/- 7.47 cm. Pathological findings were six benign or borderline phyllodes tumours (66.7%) and three cystosarcomas phyllodes (33.3%). The treatment consisted in simple mastectomy in seven cases (77.8%) and tumorectomy in two cases (22.2%). All the surgical margins were clear. Two of the three cystosarcomas phyllodes received adjuvant external bean radiation therapy 50 Gy on the thoracic chest wall. After a median outcome of 3 +/- 2 years ranging from 3 to 74 months, we did not note any relapse or metastasis. DISCUSSION AND CONCLUSION: In our series, PTB happened exclusively in females. History of fibroadenoma within 22.2% of the patients suggests the hypothesis of a filiation between these two entities. Their distinctive features were young age in diagnosis, long delay before consulting, important tumor size, predominant benign and borderline histological types, treatment mainly surgical and good local and distant control.