Primary hyperoxaluria detected by bone marrow biopsy: case report.

BACKGROUND: Primary hyperoxaluria is a rare disease with an estimated prevalence of 1 to 3 cases per million. It is due to a hepatic enzyme deficiency responsible for an endogenous overproduction of oxalate. Oxalate crystals commonly deposit in the kidney and more rarely in bone marrow. The literature has reported, to the best of our knowledge, only two cases of hyperoxaluria diagnosed by bone marrow biopsy and our case is the only one that does not show radiological bone lesions. CASE PRESENTATION: A young 22 year old chronic hemodialysis patient with nephrocalcinosis. The patient had a personal and family history of recurrent kidney stones. He presented bone pain with worsening of his general state. On physical examination, no organomegaly was detected. Biological check-up showed only a normochromic and normocytic regenerative anemia resistant to treatment and a bone marrow biopsy was performed. It showed deposits of crystals of oxalate in the bone marrow surrounded by inflammatory reaction against foreign bodies. Given our context, no liver biopsy or genetic studies, which are gold standard of diagnosis testing, were done. The diagnosis of primary hyperoxaluria was made based on morphological characteristics of crystals, his medical and family history, and the absence of any secondary cause of the condition. Since curative treatment is not available in our country, the patient only receives a palliative treatment. CONCLUSION: Primary hyperoxaluria is rarely evoked by the histological study of a bone marrow biopsy. The lack of the possibility of the only effective treatment in our context and the diagnosis, usually late, of this pathology are at the origin of the fatal evolution of the disease in almost all the cases.

[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.

Rare variant of metaplastic carcinoma of the breast: a case report and review of the literature.

BACKGROUND: Metaplastic carcinoma encompasses a group of neoplasms characterized by differentiation of the neoplastic epithelium into squamous cells and/or mesenchymal-looking elements. Spindle cell carcinoma is a rare variant of this special histological type. Its prognosis remains poor, with a high rate of local recurrence and distant metastasis. To date, only a small number of cases have been described. There is no clear agreement on this histological subtype. CASE PRESENTATION: We report a case of a 53-year-old Moroccan woman who consulted our institution following palpation of a nodule of the left breast. Mammography in combination with breast ultrasonography revealed a lesion classified as Breast Imaging Reporting and Data System 4 with microcalcification. The patient was diagnosed with spindle cell carcinoma of the breast. The diagnosis was based primarily on histological and immunohistochemical studies of the breast biopsy and secondarily on the surgical specimen. No local or distant metastasis was found. The treatment used was total surgical excision followed by radiotherapy. CONCLUSIONS: We describe the features (epidemiological, clinical, histological, immunohistochemical, and therapeutic outcomes) of our patient's case and compare them with literature data.

Lateral cervical thymic cyst in a child: a case report.

BACKGROUND: Cervical thymic cysts are uncommon lesions, rarely considered in the differential diagnosis of neck cysts in children. CASE PRESENTATION: We report a rare case of multiloculated thymic cyst in an 8-year-old boy on the right side of the neck. Perioperative diagnosis was a cystic hygroma. Macroscopic examination showed a cystic mass measuring 6.5 cm in total length. Histopathology of the excised specimen revealed thymic tissue with prominent Hassall's corpuscles associated with multiloculated cyst. The cyst wall is bordered by a flattened or multilayered epithelium, often abraded. CONCLUSION: This case is presented here for its rarity and should be included in the differential diagnosis of neck masses in children. So, it's a lesion to be well aware of, particularly by pathologists.

Round cell sarcoma of the colon with CIC rearrangement.

BACKGROUND: The CIC-rearranged sarcoma is a very rare highly aggressive malignant soft tissue group of tumors. It has recently been described as highly aggressive soft tissue tumors of children and young adults sharing similar morphological features with the Ewing sarcoma. The digestive localization is exceptional. CASE PRESENTATION: A 14-year-old male presented with a history of abdominal pain for 1 year, which increased in intensity over the last 2 months. Imaging findings showed a large heterogeneous mesenteric mass on the left flank of the abdomen. Exploratory laparotomy was performed and revealed a large cystic hypervascularized mass depending on the transverse colon and mesocolon. A wide excision of the lesion was performed with segmental colectomy. No postoperative complications were noted. The microscopic examination revealed a vaguely nodular growth of undifferentiated small round cells, arranged in solid sheets separated by thin fibrous septa with a scarce stroma. After an uncomplicated post-operative course, the patient was referred for chemotherapy. The patient died 2 months later with a peritoneal and pleural progression. CONCLUSIONS: The CIC-rearranged sarcoma is an aggressive tumor. There is no standard therapy for this rare disease. Their treatment includes surgery and chemotherapy. Resistance to chemotherapy is common. Further publications and studies will help to determine a standard therapy for this rare disease.

Two years survival of primary cardiac leiomyosarcoma managed by surgical and adjuvant therapy.

BACKGROUND: Cardiac tumors are a very rare entity. Leiomyosarcoma represents less than 1% of cases. CASE PRESENTATION: a 51-year-old woman diagnosed with primary left atrium leiomyosarcoma. She was treated by optimal surgery and adjuvant chemotherapy. She is still alive after a follow-up of 24 months without evidence of local or distant recurrence. CONCLUSIONS: Cardiac leiomyosarcoma is a rare tumor with a dismal prognosis. Surgery is the mainstay of treatment. Adjuvant treatment is still controversial.

[Cutaneous cryptococcosis mimicking basal cell carcinoma and revealing systemic involvement in acquired immunodeficiency]. / Cryptococcose cutanée mimant un carcinome basocellulaire et révélant une atteinte systémique sur immunodéficience acquise.

BACKGROUND: Cryptococcosis is a rare and a serious opportunistic infection that occurs primarily on the field of immunodeficiency. We report a case of disseminated cryptococcosis in acquired immunodeficiency syndrome revealed by unusual skin lesions. OBSERVATION: A 52-year-old patient consulted for two crusty ulcerative lesions situated on the left supraorbital and on the nasal tip that appeared 6 months ago. He also reported respiratory symptoms present since one year, with dry cough and dyspnea, chronic headache and vomiting with no alteration in visual acuity. The mycological study of the skin biopsy on both lesions isolated Cryptococcus neoformans as well as in the sputum and cerebrospinal fluid. Serology of human immunodeficiency virus infection was positive. Treatment with fluconazole, local care and antiretroviral triple therapy was implemented. DISCUSSION: Skin lesions during cryptococcosis are rare and observed in 2-10% of cases. Cutaneous symptoms were the reason for consultation in our patient. This is a rare form of cutaneous cryptococcosis leading to the discovery of both pulmonary and central nervous system locations, and to diagnosis of HIV infection.

Primary osteosarcoma of the cervical spine: one case.

The authors report the case of a 31-year old woman who was operated upon for cervical bone tumour. The pathological study showed that it was a primary, highly differentiated osteosarcoma. The clinical picture was limited to a spinal cord compression syndrome without signs of inflammation. Radiological findings were non-specific. Treatment consisted of laminectomy, radiotherapy and chemotherapy. The course of the disease was marked by stabilization, then rapid aggravation 2 years after the operation. A review of the literature highlighted the rarity and relatively slow progression of primary osteosarcomas of the spine.

[Dispensation of opioids by pharmacists in Tunisia]. / Dispensation des opioïdes par les pharmaciens en Tunisie.

This national survey aims to evaluate opioid availability and prescription by pharmacists in Tunisia and to examine pharmacists' attitude regarding Tunisian law. We surveyed a sample of 300 pharmacists randomly selected from the National Council of Pharmacists list and using the random table. This study started in September 1999 by sending to pharmacists a confidential questionnaire asking about the importance, the rate of sale and the availability of analgesics in their pharmacy. It also tried to determine pharmacists' opinions regarding Tunisian law. A total of 157 pharmacists out of 300 responded to the survey (52 per cent), 95 per cent were working in pharmacies and 15 per cent in hospitals. Analgesics were estimated to be important to very important in their work in 84 per cent and less important in 16 per cent of cases. They were given under advice with great importance in 85 per cent of cases and with less importance in 15 per cent of cases. Analgesic self-medication was frequent in 95 per cent of cases and rare in 4 per cent of cases. Analgesics of levels 1 and 2 were often to always available in 97 per cent of cases and rarely available in 1.5 per cent of cases. Some 84.7 per cent of pharmacists had opioid supplies and 8.9 per cent had no opioids in stock and 6.4 per cent hadn't given a response. 30 per cent of pharmacists think that the 7 days law for opioid prescription should be modified and 66 per cent think it should not, fearing illicit use, fraud and dependance. Pharmacists think that the minimal list of opioids to be stocked in a pharmacy is sufficient because of low demand.

[Radiological diagnosis of calcifications of the portal vein. Apropos of a case]. / Diagnostic radiologique des calcifications de la veine porte. A propos d'un cas.

The very rare portal vein calcifictions are detected in patients with a portal hypertension syndrome (PHT). A patient with hepatosplenomegaly and mild signs of PHT presented images of parietal calcifications, and the diagnostic values of different imaging techniques, particularly ultrasound and CT scan, are compared.

[Osteoarticular manifestations of sickle cell anemia. Update apropos of a series of 29 cases]. / Manifestations ostéoarticulaires de la drépanocytose. Mise au point à propos d'une série de 29 cas.

In a retrospective study, the authors report 14 cases of osteoarticular manifestations, among 29 patients affected with a sickle cell disease observed at adult age. They insist on their frequency and their interest for diagnosis and prognostic. Effectively, the diagnosis has been revealed on occasion of these manifestations in 9 cases/14. The articular signs are noted in 35%, and the osseous signs in 25% of cases.

[Epidemiology of urinary lithiasis]. / Epidémiologie de la lithiase urinaire.

The objective of this study was to analyse the epidemiological profile of urinary stones based on one thousand cases observed in our institution over a 10-year period. The mean age of the patients was 45 years and two-thirds of patients were males. The kidney was the commonest site of stones, in 57.8% of cases. The stone was radiopaque in 86.4% of cases and was a staghorn calculus in 12.2% of cases. An associated renal malformation was observed in 10.4% of cases. Urinary stones is therefore a common disease, essentially observed in a young population and characterized by recurrence. It therefore constitutes a public health problem and prevention consists of detecting recurrences and treating stone-inducing factors.

[Prostatic abscesses. A review]. / Les abcès prostatiques. Mise au point.

We review the literature to the diagnosis and therapeutic aspect of prostatic abscess. The prostatic abscess having become an uncommon disease. The diagnosis of prostatic abscess has been nearly made by transrectal ultrasound and computed tomography scan. The best diagnostic method is considered to be the transrectal ultrasound. The choice therapy was intravenous antibiotic, and drainage by ultrasound guided transperineal percutaneous puncture.

[An unusual cause of renal colic. An osteophytic spur of L3, surgically treated with success]. / Une cause inhabituelle de colique néphrétique. Un cas de bec ostéophytique de L3, opéré avec succès.

The authors report an unusual case of renal colic occurring in a 44-year-old docker. Intravenous urography showed right ureteral extrinsic compression by an osteophyte of the 3rd lumbar vertebra. After failure of medical treatment, the patient was operated with resection of the osteophyte. The postoperative course was uneventful with reduction of pain.

[Hydatid cyst of the psoas muscle. Apropos of a case]. / Kyste hydatique du psoas. A propos d'un cas.

The authors report a rare case of hydatid cyst of the psoas muscle in a 28-year-old woman, operated for vertebrospinal hydatid cyst. The patient presented with a left iliac fossa mass. Ultrasonography and computed tomography contributed to the preoperative diagnosis, despite negative hydatid serology. A retroperitoneal approach by left lumbotomy with partial pericystectomy was performed. In the light of this case, the authors discuss the diagnostic and therapeutic problems raised by hydatid cyst of the psoas muscle.

[Primary pyogenic abscess of the psoas muscle. Apropos of 5 cases]. / Les abcès primitifs à pyogène du psoas. A propos de 5 cas.

The authors discuss the diagnostic and therapeutic aspects of primary pyogenic abscess of the psoas muscle based on a series of 5 cases. Five patients between the ages of 15 and 64 years (mean: 38.2 years) were admitted to the urology department between January 1994 and December 1996 with fever, abdominal pain and low back pain, and psoitis in 1 case. The mean delay to consultation was 42 days. Clinical examination revealed a painful mass in the lumbar region and flank in 5 cases. Leukocytosis was detected in 5 patients. Radiological examination demonstrated loss of the lateral border of the psoas in 4 cases. Ultrasonography showed an enlarged psoas muscle in every case with a hypoechoic mass in 2 cases and a heterogeneous mass in the other 3 cases. Computed tomography, performed in 2 patients, confirmed the ultrasound findings in 1 case and excluded the diagnosis of type IV hydatid cyst of the psoas muscle in the other patient. The abscess was evacuated by percutaneous drain in 1 patient requiring a second percutaneous drainage for reconstitution of the abscess. Surgical drainage was performed immediately in 3 patients and after failure of antibiotic treatment in 1 patient. The hospital stay did not exceed 7 days after surgical drainage, but was 25 days after the first percutaneous drainage and 20 days after the second percutaneous drainage. The authors emphasize the multiple advantages and efficacy of surgical drainage of psoas abscess.