Unexpected infant death secondary to a pulmonary infiltration due to acute myelocytic leukaemia.

Acute myeloid leukaemia (AML) often presents with non-specific symptoms such as fatigue, anaemia or infection. Pulmonary involvement is uncommon in AML during the course of the disease and is usually caused by infection, haemorrhage, leukaemic pulmonary infiltrates and leukostasis. Lung localization of AML is very uncommon and potentially life threatening if not diagnosed and treated rapidly. The authors describe the sudden death of an asymptomatic five-month-infant because of a misdiagnosed lung localization of AML. Autopsy examination followed by histopathological studies showed an extensive leukostasis and extramedullary leukaemic infiltrating the lungs. Special stains and immunohistochemical studies revealed findings consistent with acute myelogenous leukaemia. This case suggests that underlying acute leukaemia should be considered as a cause of flu-like symptoms in infants. Medical personnel are urged to be alert to fever, sore throat, weakness and dyspnea that may be characteristic of serious systemic diseases.

[About a rare tumor of the kidney: Malignant mixed epithelial and stromal tumor]. / À propos d'une tumeur rarissime du rein: la tumeur mixte épithéliale et stromale maligne.

Mixed epithelial and stromal tumor of the kidney is a recently described neoplasm that predominantly affects perimenopausal women. Few cases with malignant features have been reported. Here, we report the case of malignant mixed epithelial and stromal tumor of the kidney with sarcomatous transformation arising in a 27-year-old female. She presented with abdominal discomfort. Computed tomography of the abdomen revealed a large compressive mass arisen from the left kidney with solid and focal cystic components. The patient underwent left radical nephrectomy. Histologic sections showed benign and malignant components. The benign component consisted of multiple tubules and variably sized cysts lined by benign epithelium. The malignant component was composed of undifferentiated cellular spindle cell sarcoma. By immunohistochemical studies, the epithelial component was positive for cytokeratins and epithelial membrane antigen (EMA). The stromal component displayed strong immunohistochemical expression of vimentin, CD99, bcl2; and was negative for cytokeratins, desmin, SMA, S-100, estrogen receptor (ER) and progesterone receptor (PR). Analysis by reverse transcriptase polymerase chain reaction (RT-PCR) failed to identify the SYT-SSX1 or SYT-SSX2 fusion transcripts characteristic of synovial sarcoma.

[Primary cutaneous osteosarcoma]. / Ostéosarcome cutané primitif.

BACKGROUND: Osteosarcoma is a malignant tumor of mesenchymatous origin that chiefly affects the metaphysis of long bones. The extraskeletal form of the disease is rare, and even rarer is a cutaneous site, whether metastatic or primary. Herein, we report a new case of primary cutaneous osteosarcoma. PATIENTS AND METHODS: A 54-year-old woman presented a hard subcutaneous nodular tumor on her left arm noted 1 year earlier. The diagnosis of cutaneous osteosarcoma was made on the basis of histological analysis of the lesion, which showed a sarcomatous dermal-hypodermal proliferation secreting osteoid. Clinical and radiological staging ruled out any extra-cutaneous spread, particularly to bone, thus confirming the primary cutaneous nature of the osteosarcoma. DISCUSSION: Primary cutaneous osteosarcoma is a rare tumor, diagnosis of which is normally based on histopathological features.

[Paraganglioma of spermatic cord]. / Paragangliome du cordon spermatique.

Paragangliomas can exist anywhere in the distribution of neural crest derived cells. In the spermatic cord, they are exceedingly rare. We report a case of histologically discovery, occurring in a man of 50 years who had a para-testicular mass, painless, firm without other signs associated. In view of this observation and a review of the literature, we propose to establish the epidemiological profile and study the prognostic and the clinicopathologic features of this tumor.

Acute generalized exanthematous pustulosis induced by the essential oil of Pistacia lentiscus.

Acute generalized exanthematous pustulosis (AGEP) is an uncommon pustular eruption characterized by small nonfollicular pustules on an erythematous background, sometimes associated with fever and neutrophilia. Over 90% of cases are drug-induced; however, it can be caused in rare cases by other agents. We report two cases of AGEP secondary to ingestion of Pistacia lentiscus essential oil, the first two such cases to our knowledge. The cutaneous morphology, disease course and histological findings were consistent with a definite diagnosis of AGEP, based on the criteria of the EuroSCAR study group. These two cases highlight the need to consider herbal extracts as a potential rare cause of AGEP and to ensure the safety of herbal medicines.

[Ileocecal ameboma: a case diagnosed by pathology and molecular biology]. / Amoebome iléocaecal: un cas diagnostique par l'anatomie pathologique et la biologie moléculaire.

Colonic ameboma is a rare benign inflammatory tumor due to the infection by Entamoeba histolytica and poses frequently the problem of colon cancer. We report a case of a 52 year-old patient who presented a cecal amoeboma revealed by a painful mass in the right iliac fossa. Radiologic and endoscopic examinations depicted a parietal thickening of the right colon and the cecum. A presumptive diagnosis of colon cancer was firstly discussed. Confirmation of ameboma was made on pathological examination, PCR and serology.

[Erythematous type scaly papule on the abdomen: chromoblastomycosis due to Fonsecaea pedrosoi]. / Plaque érythémato-squameuse papuleuse et atrophique de l'abdomen: chromomycose à Fonsecaea pedrosoi.

Chromoblastomycosis is a chronic subcutaneous fungal infection caused by dematiaceous saprophytic moulds. We report a case of chromoblastomycosis due to Fonsecaea pedrosoi observed in man from the Baja region of Tunis. He presented since one year an erythemato-squamous atrophic plaque localised at the abdomen area. Clinical remission was obtained after cryotherapy and terbinafine.

[Paraparesis and fever in a Tunisian woman: cryptococcal spondylitis with spinal involvement]. / Paraparésie fébrile chez une Tunisienne: spondylite à cryptocoque avec atteinte médullaire.

Cryptococcus neoformans is a ubiquitous yeast that causes opportunistic infections mainly involving the central nervous system. Cryptococcoma is a rare entity characterized by a solid, tumor-like mass that is usually located in the cerebral hemispheres or cerebellum. Spinal involvement is rare with only 6 cases reported in literature. Bony involvement is also a rare occurrence that has been observed in only 5 to 10% of reported cases of infection by Cryptococcus neofomans. The purpose of this report is to describe a case of paraplegia due to cryptococcal spondylitis with spinal cord involvement in an HIV-seronegative patient with a history of systemic sarcoidosis. Diagnosis was achieved by histological examination of the surgical specimen.

[Pyoderma gangrenosum and systemic lupus erythematosus: A rare association]. / Pyoderma gangrenosum et lupus érythémateux systémique : une association rare.

INTRODUCTION: Pyoderma gangrenosum (PG) is a neutrophilic dermatosis that is traditionally associated with systemic disorders such as chronic inflammatory bowel diseases, rheumatoid arthritis and malignant hematologic disorders. Its association with systemic lupus erythematosus (SLE) is rare and not well known. We report a case of this association with a review of the literature. CASE REPORT: A 43-year-old female patient, followed for 4 years for SLE, presented a deep ulceration of the anterior face of the left thigh with inflammatory borders, an ulcerated nodule of the right shoulder and four small ulcerations of the back of the right hand. The biopsy of the ulceration of the left thigh concluded to PG. The patient was treated by corticosteroids with complete healing of lesions. CONCLUSION: The prognosis of lupus does not seem to be aggravated by PG and the treatments of a SLE flare are usually enough for treating associated PG.

[Epidemiology of extrapulmonary hydatid cysts: 265 Tunisian cases]. / Epidémiologie des kystes hydatiques extrapulmonaires : 265 cas en Tunisie.

AIM OF STUDY: The authors wanted to analyze the epidemiological features of extrapulmonary hydatid cysts and compare their results with those reported in literature. METHODS: Two hundred and sixty-five cases of extrapulmonary hydatid cysts collected from 1990 to 2007 were retrospectively studied. RESULTS: One hundred and one male and 164 female patients (sex ratio M/F=0.61 mean age 38.7 years) were included. In our series, hydatid cysts involved mainly the kidney (24.1%), the central nervous system (22.6%), the liver (19.6%) and the spleen (11.3%). CONCLUSION: Contrary to published data, our results show that hydatid cysts of the kidney and of the central nervous system are more frequent than hepatic location which ranks 3rd. Such unusual results may be due to a selection bias.

[Ulcerations of the extremities: An unusual manifestation of Behçet's disease]. / Ulcérations des extrémités : une manifestation inhabituelle de la maladie de Behçet.

BACKGROUND: Although cutaneous and mucosal involvement is a major manifestation in Behçet's disease, ulcerated lesions of the extremities are exceptional and poorly known. CASE REPORT: A 57-year-old male patient was diagnosed 7 years ago with Behçet's disease. This diagnosis was made in the presence of recurrent bipolar aphtous ulcers, pseudofolliculitis lesions and retinal vasculitis. After having been lost to follow up for two years, during which his treatment was discontinued, he presented centimetric necrotic ulcerations of the fingers of the hand and of the right big toe. The biopsy revealed leucocytoclastic and necrotizing vasculitis. The patient improved with antibiotic, oral corticosteroids, colchicine and local care. CONCLUSION: Linking extremity ulcers with Behçet's disease, though sometimes difficult, is essential for proper management.

[Pulmonary hyalinizing granuloma revealed by a pituitary gland diffusion]. / Granulome pulmonaire hyalinisant révélé par une localisation à la base du crâne.

INTRODUCTION: Pulmonary hyalinizing granuloma is a rare fibrosing lesion of the lung, characterized by its histological appearance which includes central whorled deposits of lamellar collagen. The extrapulmonary diffusion of the disease is extremely rare, and in our knowledge any case of pituitary diffusion has ever been reported in the literature. EXEGESIS: We reported an unpublished case of a 31-year-old woman presenting with amenorrhea, galactorrhea, diplopia, headache, polyuria and polydipsia. The diagnosis of pulmonary hyalinizing granuloma revealed by an intracranial localization was based on radiologic and pathologic findings. Clinical course was favourable with corticotherapy. CONCLUSION: Our report is particular because cerebral localization was the initial manifestation of primary hyalinizing granuloma and because of the favourable outcome with corticotherapy.

Supernumerary nipples in association with Hailey-Hailey disease in a Tunisian family.

Supernumerary nipples (SNs) or polythelia are developmental abnormalities of breast tissue. They are located along the embryonic mammary lines. Polythelia usually occurs as a sporadic abnormality, although familial aggregation has been occasionally reported. Hailey-Hailey disease is a rare autosomal genodermatosis characterized by disturbed keratinocyte adhesion. These cutaneous disorders have been described in correlation with many other abnormalities. We report here the association of Hailey-Hailey disease and supernumerary nipples in a Northern Tunisian family. To our knowledge, this is the first report of such a clinical association.

Hemangiopericytoma in the central nervous system. A study of eight cases.

Most hemangiopericytomas (HPC) are located in the musculoskeletal system and the skin, while the location in the central nervous system (CNS) is rare. The latter represents 2 to 4% in large series of meningeal tumors, thus accounting for less than 1% of all CNS tumors. In the central nervous system, tumors with a hemangiopericytomatous histolopathological pattern can be either hemangiopericytomas or solitary fibrous tumors. CNS-HPCs have a relentless tendency for local recurrence and metastases outside the CNS. Metastasis can also appear many years after adequate treatment of the primary tumor. We present a pathological study of eight patients with CNS-HPC and compare our results with corresponding published data. The CNS-HPC group consisted of three males and five females with a mean age of 36.75 years. The tumors were supratentorial in four cases, infratentorial in two cases, tentorial in one case and located in the spinal cord in the last one. Histologically, CNS-HPCs were similar to their soft tissue counterparts. One case demonstrated increased cellularity, marked nuclear hyperchromasia and marked cellular pleomorphism with infiltration of the cerebellum. All patients underwent surgery with gross-total resection in all cases. No patients received postoperative radiation therapy. Only four patients recurred locally after six, seven and eight months, and five years. Our study presents the pathological features of CNS-HPC as a distinct entity from both meningioma and solitary fibrous tumors. A comparative review of literature with our results is discussed.

[Kaposi sarcoma in a patient with ANCA vasculitis requiring hemodialysis]. / Sarcome de kaposi compliquant une vascularite à ANCA en hémodialyse chronique.

INTRODUCTION: Iatrogenic Kaposi's sarcoma is widely reported after transplantation. Less commonly, it occurs in patients receiving immunosuppressive therapy for ANCA associated vasculitis. We report here the rare association of Kaposi's sarcoma, prurigo nodularis and ANCA associated vasculitis in a hemodialysis patient. CASE REPORT: We describe a 58-year-old woman who presented granulomatosis with polyangeiitis with alveolar hemorrhage and renal failure requiring hemodialysis. She developed cutaneous Kaposi's sarcoma seven weeks after the beginning of immunosuppressive therapy. Biological tests showed negative HHV8 virus infection. Lesions of Kaposi's sarcoma responded to a discontinuation of immunosuppressive drugs and a decreasing dosage of corticosteroids. CONCLUSION: Our case showed that the immunosuppressed state related to multiple factors such as underlying disease, immunosuppressive therapy and hemodialysis may all have contributed to the development of this neoplastic disorder in our patient.

[Benign sacrococcygeal teratoma in an adult]. / Tératome sacrococcygien bénin de l'adulte.

Sacrococcygeal teratomas are rare congenital tumors generally discovered at birth. Exceptionally these tumors are observed in adults. Radical resection must be performed to avoid potentially malignant recurrence even if the primary lesion was benign. We report a case of a benign sacroccygeal teratoma diagnosed in a 25-year-old male and discuss clinical features, pathological considerations and treatment.

[Chordoid meningioma. Two cases]. / Le méningiome chordoïde. A propos de deux cas.

Chordoid meningioma is an uncommon histopathological variant of meningioma frequently confused with chordoma. Recurrence is not uncommon. The aim of our study is to present the clinical and pathological characteristics of this entity and a review of the literature. We report two cases of chordoid meningioma occurring in two women aged 47 and 63 years. Both women presented neurological signs without systemic disease. Radiological findings were temporoparietal and temporofrontal lesions with intense enhancement after contrast injection. Complete surgical resection was performed. Histopathologic findings were consistent with chordoid meningioma.

[Hydatid cyst of the posterior fossa]. / Kyste hydatique de la fosse postérieure.

Hydatidosis is an endemic affection in Tunisia. Cerebral echinococcosis is a relatively rare entity accounting for only 1-2% of all hydatid cysts in humans. Extradural hydatid cyst of the posterior fossa is a very uncommon site for the disease. We report the case of a four-year-old child admitted for high intracranial pressure. Brain CT scan showed an extradural posterior fossa cyst without enhancement after contrast medium injection. Operative finding revealed a hydatid cyst. The histological examination of the tissue sample confirmed the diagnosis. The patient was given albendazole post operatively. She feels well six months later.

[Subcutaneous phaeomycotic cyst of the ankle]. / Kyste phaeomycotique sous-cutané de la cheville.

Phaeohyphomycosis is a rare mycotic opportunist disease witch usually affects immunodepressed patients. Infection follows a traumatic inoculation of fungi into the skin with formation of a subcutaneous cyst. We report the case of a 20-year-old woman presenting with a subcutaneous, painless, and recurrent cystic lesion on the right ankle for 9 years. A surgical excision was performed. Histopathologic examination showed a granuloma with central abscess surrounded by a thick fibrous tissue. After staining with PAS, septate hyphae and spores were found in the granuloma, consistent with a phaeomycotic cyst.

[Testicular plasmocytoma: a case report]. / Le plasmocytome testiculaire: a propos d'un cas.

BACKGROUND: Extramedullary plasmocytoma is rare, with less than 50 reported cases and only 8 cases of primary testicular plasmocytoma. AIM: Report of a new case CASE: This report concerns a 65-year-old patient presenting an enlargement of the testis. The diagnosis of testicular plasmocytoma was made on histological examination. There was no evidence of bone marrow involvement or systemic signs of myeloma. This case will be studied with a review of the literature.