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.

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.

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.

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.

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.

[Lung adenocarcinoma with intramedullary spinal cord metastasis: a case report and review of the literature]. / Adénocarcinome pulmonaire avec métastases intramédullaires: à propos d'un cas et revue de la littérature.

Intramedullary spinal cord metastases are extremely rare and affect 0.1 to 0.4% of all cancer patients. We report the case of intramedullary metastases in a 42-year-old patient, 12 months after the initial diagnosis of a lung adenocarcinoma. The patient was on third-line chemotherapy for a progressive disease with node and bone metastases. He complained of weakness and difficulty walking. The MRI permitted confirmation of the diagnosis. Corticotherapy was started and the patient underwent radiotherapy. A targeted therapy was indicated. The patient died three months after the initiation of this treatment. The aim of this paper is to report a new case of intramedullary spinal cord metastases from a pulmonary adenocarcinoma, focus on its rarity and diagnostic and therapeutic difficulties through a review of the literature.

[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.

[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.

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.

[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.

[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.

[Pyonephrosis: diagnosis and treatment: report of 14 cases]. / Pyonéphrose: diagnostic et traitement: à propos de 14 cas.

Prior to the introduction of antibiotics, the treatment of pyelonephrosis frequently consisted of nephrectomy to remove the non-functional kidney, which was a potentially dangerous source of systemic infection. This approach was later modified as a result of the advances made in antibiotic therapy, and included vigorous antibiotic treatment and prompt drainage of the kidney. At present, percutaneous nephrostomy provides a means of draining off the pus and determining a possible residual renal function. In this study, 14 cases of pyonephrosis were observed over a 7-month period. Lumbar pain was noted in 70% of cases, painful lumbar contact in 5 cases and fever, shivering and pyuria in all cases. Cytobacteriological urine analysis showed the presence of Escherichia coli in 7 patients, Proteus in 4 patients, and in 3 cases abacterial leucocyturia; in 2 patients with only one functional kidney, renal insufficiency was observed. In all cases, the diagnosis was confirmed by ultrasonography. The main etiological factors were urinary lithiasis in 10 patients (71%), followed by uropathy of the pyeloureteral junction in 4 patients (29%). Treatment consisted of primary nephrectomy in 10 cases; in 3 cases, primary nephrostomy was performed with a positive outcome and recovery of renal function in 2 subjects; in one case of renal failure treated by nephrostomy followed by conservative surgery, the patient did not survive. In conclusion, nephrectomy is advocated as the treatment of choice in the case of a damaged kidney and a normal contralateral kidney. Conservative treatment should be envisaged particularly in the case of a single kidney, or if the patient's state of health is poor. The best treatment consists of the detection and cure of the lithiasis which is the main etiological factor in this pathology.

[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.

[Perineal recurrence of resected penile cancer. A case report]. / Récidive périnéale d'un cancer de la verge opéré. A propos d'un cas.

The authors report an unusual case of perineal recurrence of squamous carcinoma of the penis in a 50-year-old, white man, circumcised during infancy and operated 5 years previously for stage T3N0M0 squamous carcinoma of the penis by total amputation of the penis. Clinical examination revealed a very large, infected perineal tumour associated with bilateral inguinal lymphadenopathy. This stage T4N2M0 tumour was treated palliatively by cystostomy, emasculation with perineal tumour reduction and antibiotics. The patient became afebrile and the infection resolved and was referred to the radiotherapy department for further treatment.