[Profuse sarcoidosis associated with multiple cutaneous squamous cell carcinomas]. / Sarcoïdose profuse associée à des carcinomes épidermoïdes cutanés multiples.

BACKGROUND: The cutaneous signs of sarcoidosis are polymorphous and occasionally misleading. Herein, we report a rare case of profuse sarcoidosis involving various rare cutaneous signs specific to the disease associated with multiple cutaneous squamous cell carcinomas. PATIENTS AND METHODS: A seventy-three-year-old man had been presenting dry ichthyosiform erythroderma for 2 years. The examination also revealed diffuse cutaneous atrophy with ulceration, superficial adenopathy and pseudotumoral hypodermic masses. Ophthalmological examination revealed conjunctival nodules. Biopsy samples for the various cutaneous lesions, salivary glands, conjunctival nodules, a hypodermic nodule and an adenopathy yielded similar images evocative of epithelioid granulomas and giant cells without caseous necrosis. Infectious causes were ruled out. Respiratory function tests showed a restrictive respiratory disorder. ACE levels were extremely high. Proteinuria was noted for 24-hour urine tests and renal punch biopsy showed an appearance of membrane-proliferative glomerulonephritis. Oral corticosteroids produced clinical and laboratory improvement. Furthermore, the patient was also presenting several invasive cutaneous squamous cell carcinomas in a setting of marked helioderma. DISCUSSION: Erythrodermic, ichthyosiform, atrophied, ulcerated and pseudotumoral forms of cutaneous sarcoidosis are very uncommon and their simultaneous appearance in the same patient is extremely rare. Further, the existence of numerous cutaneous carcinomas in this patient raises the question of an association between sarcoidosis and cancer, with fortuitous association appearing unlikely.

[Diffuse tenosenovial giant cell tumor of the wrist revealed by carpal tunnel syndrome: report of a case]. / Tumeur ténosynoviale à cellules géantes diffuse du poignet révélée par un syndrome du canal carpien: à propos d'un cas.

Giant cell tumour of tendon sheath is a benign proliferative lesion of synovial origin that may affect the joints, bursae and tendon sheaths. It is the second most common soft tissue tumor of the hand after ganglion cyst. The localised (nodular) form is the most common. However, the less-common diffuse-type giant cell tumour is usually located in the peri-articular soft tissue. The authors report the case of a giant cell tumor of the tendon sheath arising from the carpal tunnel of the wrist in a 42-year-old woman. The patient presented a mild carpal tunnel syndrome and a mid-palmar swelling. We present an unusual localization of giant cell tumor of the tendon sheath, causing carpal tunnel syndrome.

[Myxoid neurofibroma of the thumb]. / Neurofibrome myxoïde du pouce.

UNLABELLED: Myxoid neurofibroma (MN) is a benign tumor of poorly identified perineural cell origin. We report a case of NM of left thumb and discuss the main differential diagnosis. CASE REPORT: A 34-year-old woman presented with a painless, slowly progressive tumor of the left thumb of 6 months duration. This tumor had recurred after resection done 26 years before. Clinical examination revealed a tumor on the anterior surface of the left thumb, painless, firm, 4×3cm, ulcerated and covered with thin telangiectasia. The X-ray of the phalanx showed no alteration to the underlying bone. The histopathological study of a skin biopsy reported a myxoid neurofibroma. Excision of the lesion was performed with preservation of the nail. Neither the patient nor her family members had neurofibromatosis. The outcome was favorable and no recurrence was noted after 18 months of follow-up. COMMENTS: The usual sites of the MN are the face, shoulders, arms, and periungual regions. It is usually a solitary lesion; however, lesions may be multiple or recur after initial incomplete excision as in our patient. It must be considered in the differential diagnosis of tumors of the extremities. We report this case because of the rarity of both the tumor and its site.

Atypical celiac disease: from recognizing to managing.

The nonclassic clinical presentation of celiac disease (CD) becomes increasingly common in physician's daily practice, which requires an awareness of its many clinical faces with atypical, silent, and latent forms. Besides the common genetic background (HLA DQ2/DQ8) of the disease, other non-HLA genes are now notably reported with a probable association to atypical forms. The availability of high-sensitive and specific serologic tests such as antitissue transglutuminase, antiendomysium, and more recent antideamidated, gliadin peptide antibodies permits to efficiently uncover a large portion of the submerged CD iceberg, including individuals having conditions associated with a high risk of developing CD (type 1 diabetes, autoimmune diseases, Down syndrome, family history of CD, etc.), biologic abnormalities (iron deficiency anemia, abnormal transaminase levels, etc.), and extraintestinal symptoms (short stature, neuropsychiatric disorders, alopecia, dental enamel hypoplasia, recurrent aphtous stomatitis, etc.). Despite the therapeutic alternatives currently in developing, the strict adherence to a GFD remains the only effective and safe therapy for CD.

[Hydatid cyst of the scalp]. / Kyste hydatique du cuir chevelu.

INTRODUCTION: Hydatid disease is a broad-based anthropozoonosis common to humans and several mammal species. The disease results from the development of Echinococcus granulosis in the body. CASE REPORT: We report a new case involving a 58-year old woman hospitalized for a subcutaneous mass in the scalp with no local inflammatory signs. Radiological examination was consistent with a subcutaneous cyst. Complete surgical resection of the mass was performed. Histopathological examination demonstrated hydatid cyst. DISCUSSION: Subcutaneous localization of hydatid cyst is uncommon even in endemic zone. Diagnosis is suggested by ultrasonography and confirmed by histology.

[Blastomycosis in Morocco: imported mycosis]. / La blastomycose au Maroc: une mycose d'importation.

Blastomycosis is a rare case. We report a first Moroccan case. A 41-year-old male presented with a 6-month history of dyspnea, fever and significant chest pain associated with night sweats and weight loss. The physical examination disclosed a firm painful paravertebral mass. The chest radiograph demonstrated a left apical opacity. The thoracic scan showed parenchymal infiltration of the apico-dorsal segment of the left upper lobe with vertebral and costal lytic lesions. Surgical biopsy showed granulomatous inflammation with giant-cell intracytoplasmic inclusions. Fungal studies yielded Blastomyces dermatitidis which responded excellently to ketoconazole. Outcome has been excellent at 3.5 years months follow-up. The clinical and radiographic presentation of blastomycosis is non-specific and can be mistaken for a neoplasm. Delay in diagnosis is common.

[Myofibroma of the mandibule: a case report]. / Myofibrome de la mandibule: à propos d'un cas.

INTRODUCTION: Myofibromatosis is a rare tumor. Two forms are described, solitary and multicentric, the solitary type is more common and is localized mainly on the head and the neck, mandible involvement is rare. The recent observation of a patient with a myofibrome of the mandible has given the opportunity to conduct an analysis and review of the literature of this disease rarely encountered. MATERIALS AND METHODS: We report a case illustrating solitary myofibroma of the mandible in a 16 year old man. RESULTS: The histological diagnosis was done on the identification of the spindle-shaped tumoral proliferation and the positive expression of the anti-vimentine, anti-smooth, muscle actin anti desmin. The treatment was surgical. DISCUSSION: myofibromatosis often presents as a painless, well-circumscribed, solid nodule. Imagery is very useful to assess lesion extension and for the therapeutic followup. The diagnosis is made on anatomopathological findings and immunohistochemical assessment. The treatment of the solitary myofibromatosis is primarily surgical and its prognosis is excellent contrary to the multicentric form.

Unusual presentation of a rare tumor of the dorsal surface of the foot.

Calcifying aponeurotic fibroma (CAF) was originally described by Keasbey in 1953 as juvenile aponeurotic fibroma, most commonly occurring in the palms of the hands and soles of the feet in children and adolescents. It usually presents as a firm, painless mass without preceding trauma. We report a case of this rare condition with an unusual presentation in a 60-year-old woman affecting the dorsal surface of the foot. It is a relatively benign condition with a good prognosis following complete surgical excision. It may have a slightly increased incidence in males. The accurate diagnosis is based only on histology but it is essential to differentiate it from other sinister lesions such as fibrosarcoma that may lead to amputation.

[Juxta articular myxoma of the wrist: a case report]. / Myxome juxta articulaire du poignet : à propos d'un cas.

Juxta articular myxomas are rare tumors. The treatment is surgical with a high recurrence rate. A location proximal to the wrist joint is exceptional. We report a case of juxta articular myxoma of the wrist in a 30-year-old patient, with no recurrence four months after surgical excision.

[Paratesticular desmoplastic small round cell tumour: case report with literature review]. / La tumeur desmoplastique à petites cellules rondes paratesticulaire: à propos d'une observation avec revue de la littérature.

Desmoplastic small round cell tumors are rare aggressive cancers of adolescence and early adulthood. It has recently been separated from other small round cell tumors because of its pathological characteristics and clinical features. They are usually intra-abdominal tumors affecting young people and have classically been associated with a bad prognosis. However, in recent years there have been reports on desmoplastic small round cell tumors affecting other body regions, including the paratesticular area. We report the case of a 27-year-old male who consulted on a progressive enlargement of the right hemiscrotum. He referred no previous urological symptoms and had no systemic symptomatology. Physical examination revealed a round elastic firm 1-cm mass of the epididymis, which was excised. A computed tomography scan showed a para-aortic mass of 1cm. Histological and immunohistochemical diagnosis confirmed a desmoplastic small round cell tumor. The patient received chemotherapy. Today, 6 months after diagnosis the patient remains well and free of disease. Recent reviews on desmoplastic small round cell tumors affecting the paratesticular area have shown a better prognosis for tumors of this origin compared to abdominal ones. We should include this lesion among the differential diagnosis of paratesticular tumors, mainly in children and adolescents.

[Breast metastasis from vulvar carcinoma: case report and review of literature]. / Métastase intramammaire d'un cancer vulvaire: à propos d'un cas avec revue de la littérature.

The breast metastases resulting of vulvar carcinoma are very rare, and represent exceptionally the first manifestation of the disease. We report the case of a 42 year-old patient who underwent a treatment because of vulvar epidermoid carcinoma, right away metastatic at the level of the inguinal ganglia. The treatment consisted in a total vulvectomy with bilateral ganglial curretage, followed by external radiotherapy about the perineum and the inguinal ganglia. Three months after the end of her treatment, the patient presented with a nodula on the left outer breast with features of malignancy noticed by clinic and mammographic examination. The histologic study of the mammary biopsy showed epidermoid carcinoma of likely metastatic origin. A left Patey has been realized and confirmed the metastatic localization of epidermoid carcinoma with axillary ganglial metastasis (2N+/-7). Besides, this patient presented a right cervical ganglial parcel that the biopsy showed a metastatic localization of a vulvar carcinoma. A palliative chemotherapy type cyclophosphamid, adriblastin, cisplatine (CAP) has been admistrated during three cycles spaced out three weeks. The patient died 11 months after the supervene of the cerebral metastasis. We present this case because its rarety and to show the possibility of metastasis at the level of breast due to vulvar cancer. The clinicians must remember this possible tropism of the vulvar cancer for the breast, not only during the supervision and the complete examination as regards the disease spreading but also when the affection revealed unknown primary tumor. The diagnostic orientation is based on the mammography and the mammary biopsy. In this stage, the treatment is unfortunately palliative, the survival until a year is not more than 20%.

[Cystic meningioma. Case report and literature review]. / Méningiome kystique. A propos d'un cas et revue de la littérature.

BACKGROUND: Cystic meningioma is a rare variety of meningioma. It represents 1,6 to 10% of intracranial meningiomas, the authors report a case of intracranial cystic meningioma with a review of literature. CASE REPORT: A 46-year-old female presented with left parietooccipital headache followed by right side hemiparesis. CT scan brain showed a left parietal tumor with double solid and cystic components thought to be glioma or metastasis preoperatively. At surgery the extraaxial solid and cystic lesion had a well defined capsule that could be easily separated from the perilesional cortical surface. The tumor was totally removed. The histological study showed a cystic meningioma. CONCLUSION: Cystic meningioma is an uncommon tumor that should be considered in the differential diagnosis of brain tumors with a cystic component.

[Appendicular mucocele caused by carcinoid tumor during pregnancy]. / Mucocèle de l'appendice sur tumeur carcinoïde pendant la grossesse.

Mucocele of the appendix secondary to appendicular carcinoid tumour is a rare entity. It occurs exceptionally during pregnancy. The interaction between carcinoid tumour or mucocele of the appendix and pregnancy is not yet elucidated. Prognosis depends on the size of the carcinoid tumour and its regional invasion. We report a case of appendicular mucocele caused by a carcinoid tumour in a 36-year-old pregnant woman who had been admitted because of acute appendicitis. Pathological examination of the appendicectomy specimen revealed a carcinoid tumour measuring 2 cm. Right hemicolectomy was performed two weeks after delivery with good evolution. According to this case and depending on the literature, the authors discuss the diagnosis and the therapeutic modalities proposed for this rare entity.

[Léiomyosarcoma of the diaphragm: a case report and review of the literature]. / Le léiomyosarcome diaphragmatique: revue de la littérature, à propos d'une observation.

Leiomyosarcomas of diaphragm are exceptional. Since 1935 only eight cases have been published. We report a new case of a 18-year-old man who consulted for an abdominal mass. Ultrasonography revealed a kystic and septated mass measuring 157 mm of diameter. Tomodensitometry showed a sus-mesocolic and voluminous left tumoral mass wish was widely necrosed. The mass was displacing the stomach on the right. A widely surgical resection of tumour was performed. Diagnosis of leiomyosarcoma of diaphragm was performed by pathological study. The outcome was marked by tumour recurrence three months later. The patient died after six months. The review of literature show that prognosis of diaphragmatic leiomyosarcomas is poor.

[Sweet's syndrome in a child]. / Syndrome de Sweet chez un nourrisson.

INTRODUCTION: Sweet's syndrome was described for the first time in 1964. It is usually described in adults and remains rare in children. We report a case in a 23 month-old infant. OBSERVATION: A 23 month-old boy presented with diffuse papular, edematous annular plaques surrounded by vesicles and bullas. Laboratory examinations revealed neutrophilic polynuclear hyperleukocytosis, anemia and an inflammatory syndrome. The myelogram was rich and the abdominal sonography normal. Oral corticosteroids (2 mg/kg/d) led to spectacular improvement. After 19 days' treatment, the boy developed cervical adenopathies and hepatomegaly. The second myelogram was normal. Evolution under corticosteroids was good. DISCUSSION: Sweet's syndrome is exceptional in infants. The frequent association with a malignant blood disease should prompt appropriate investigations and prolonged surveillance. Systemic corticosteroid therapy is the reference.

[Primary malignant non-Hodgkin skeletal muscle lymphoma: a case report]. / Lymphome malin non-hodgkinien primitif du muscle squelettique: à propos d'un cas.

BACKGROUND: Malignant non-Hodgkin lymphoma (NHL) is rarely encountered in soft tissue. The differential diagnosis with other soft tissue tumors, particularly sarcoma, is difficult. CASE REPORT: A 70-year-old woman presented an increase in the volume of the right thigh. Ultrasonography visualized a hypoechogenic mass with irregular contours. At computed tomography, the mass measured 17x14x7 cm and was situated in the biceps femoris. Enhancement was heterogeneous after intravenous contrast injection with discrete infiltration of the subcutaneous fat. Tumor biopsy demonstrated diffuse malignant small B-cell lymphomatous-plasma cell proliferation. The diagnosis was primary NHL of the biceps femoris. Search for extension was negative. Wide resection was followed by chemotherapy. The patient was free of recurrence at 10 months follow-up. DISCUSSION: Primary NHL of skeletal muscle is usually observed in elderly subjects, principally in thigh muscles. Magnetic resonance imaging and computed tomography visualize characteristic features. Diagnosis is formally established by pathology.

[Idiopathic granulomatous mastitis: apropos of 8 cases and review of the literature]. / Mastite granulomateuse idiopathique: à propos de huit cas avec revue de la littérature.

AIM OF THE STUDY: The authors report eight cases of idiopathic granulomatous mastitis in which the epidemiological, clinical, diagnostic, therapeutic and prognostic features are discussed. MATERIAL AND METHODS: Retrospective study of eight cases registered among 2,000 breast anatomopathological study during 5-years period. RESULTS: The mead age was 44.3 years. Breast lump measuring 2.5 to 15 cm in size was noted at clinical examination. Mammography noted nodular opacities and ultrasonography showed hypoechoic nodules. Histological findings were consistent on granulomatous inflammation centred on mammary lobules. The inflammatory infiltrate was including leukocytes, lymphocytes, plasma cells as well as epithelioid and giant cells without caseous necrosis in the granuloma. DISCUSSION AND CONCLUSION: Idiopathic granulomatous mastitis is a rare entity. This entity can clinically mimic other breast mastitis disease. Diagnosis is assessed by histological analysis This disease can be treated with drugs and surgical excision of the lump. The prognostic of this chronic disease is favourable.