[Marcus Gunn Syndrome. Study of 3 rare cases]. / Syndrome de Marcus Gunn. Etude de 3 cas rares.

PURPOSE: reporting three rare cases of Marcus Gunn Syndrome and analyzing the clinical features of this entity. METHODS: Case reports of three patients with a Marcus Gunn Syndrome, treated at the Ophthalmology B CHU service Rabat - Morocco. RESULTS: Our patients are two boys aged 1 and 2 years old, and a 31 year old woman with a Marcus Gunn Syndrome. As special features, in the boys' cases this syndrome is bilateral, as for the other case it is associated with congenital fibrosis clinically predominant of the Ipsilateral inferior rectus muscle in an adult. Surgical treatment was not offered for children because of their young age. The adult patient has benefited from a recession of the inferior rectus muscle and a pleating in the superior rectus muscle. The forced duction test objectified a fibrosis of the inferior rectus muscle. Surgical correction of Ptosis and Synkinesis has not been indicated in this patient, given the residual oculomotor disorder and the risk of exposure keratitis. CONCLUSION: Marcus Gunn Syndrome is exceptional in adults. This can be explained by the tendency of the lid retraction to fade with age. Bilateral involvement in Marcus Gunn Syndrome is rare. Its association with congenital fibrosis of extraocular muscles has allowed a better etiopathogenic approach highlighting a supra nuclear origin of this Syndrome.

[Role of the pathologist in the diagnosis of palpebral keratoacanthoma: case report and literature review]. / Rôle du pathologiste dans le diagnostic du kératoacanthome de la paupière : à travers un cas et une revue de la littérature.

We report the case of a keratoacanthoma of the lower lid in a 14-year-old girl posing diagnostic difficulty with differentiating the lesion from a verrucous squamous cell carcinoma on histologic examination of an incisional biopsy. It was only upon complete excision of the tumor that the diagnosis of keratoacanthoma could be made and that of malignancy ruled out. We discuss the clinical and histologic diagnostic criteria for keratoacanthoma and assert that wide biopsy is essential for adequate morphologic study to enable better therapeutic management.

[Solitary fibrous tumor of the orbit: report of two cases and review of literature]. / Tumeur fibreuse solitaire de l'orbite : à propos de deux cas avec revue de la littérature.

Solitary fibrous tumor (SFT) is a rare mesenchymal neoplasm that most frequently arises in the pleura and uncommonly involves the orbit. There are 54 cases of SFT of the orbit available in the literature. The authors report tow cases of an orbital SFT and discuss the clinical, radiological, pathological features and differential diagnosis. Long-term follow-up of patients is necessary because SFT may recur many years after operation. Complete surgical resection is the most important prognosis factor of this tumor.

[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.

[Iterative surgery for goiter]. / Les réinterventions après thyroïdectomie pour goitre.

AIM: Identify and resolve problems related to iterative surgery for goiter. PATIENTS AND METHOD: Fifty-nine patients who underwent surgery for recurrent benign goiter between 1990 and 1999 were included in the study. RESULTS: Forty-two patients had lobectomy and isthmusectomy (71.2 and 17 patients had subtotal thyroidectomy (28.8%). The delay from initial surgery to recurrence was less than 5 years for 44% of the patients and was more than 10 years for 17.8%. There was no post-operative mortality and no recurrent laryngeal nerve injury. Early post-operative hypocalcemia occurred in 2 patients (3.3%). CONCLUSION: The goal of revision surgery for recurrent goiter is total thyroidectomy. Prevention is a rational management scheme for thyroid nodules.

[Tuberculosis of the thyroid. Diagnosis and treatment]. / La tuberculose de la thyroïde. Diagnostic et traitement.

Thyroid tuberculosis is rare. We present the case of a 25 year-old woman with tuberculosis of the thyroid. Although seldom observed, tuberculosis should be considered in the differential diagnosis of nodular lesions of the thyroid. Diagnosis is made by histological examination and demonstration of the tubercle bacilli from biopsy or aspiration specimen. The efficacy of fine-needle aspiration cytology in diagnosis of tuberculosis of the thyroid is proved. Administration of antituberculous drugs is considered as the treatment of choice. Abscess drainage is sufficient. In rare cases surgery is necessary. The prognosis is good.

Endoscopic ureteroneocystostomy: palliative urinary diversion in advanced prostatic cancer.

Distal ureteral obstruction is a common complication in advanced prostatic cancer, secondary to direct compression at the ureterovesical junction and/or invasion of the ureteral orifice and/or of the intramural ureter by the tumor. The ureteral orifice may not be readily visible to the trigone-infiltrating tumor, but remains superficial as it is pushed upward by the tumoral tissue. The indications for palliative diversion in patients with pelvic malignant disease and renal failure from ureteral obstruction remain controversial. We present the results of our endoscopic ureteroneocystostomy technique by which the trigone is resected to restore continuity of the ureteral orifice and to place a double pigtail ureteral stent. Of the 31 operations performed for obstructive prostate cancer, continuity was restored in 76% of cases. Length of hospital stay was gradually reduced to a few days. Median survival after surgery was 8 months (0.25 to 27.5 months). Palliative diversion may be considered a reasonable option for many patients, even in the case of an apparently unpassable obstruction of the pelvic ureter, thereby prolonging survival and improving quality of life.

[Urinary calculi and indinavir sulfate in patients with HIV infection. Apropos of 4 cases]. / Lithiase urinaire et sulfate d'indinavir chez les patients infectés par le VIH. A propos de 4 cas.

We report on 4 cases of urinary stone observed in patients treated with the drug Crixivan (Indinavir Sulfate) and review the literature. Comments include stone composition, clinical aspects, treatment and prevention.

[Retroperitoneal liposarcoma. Apropos of 2 cases]. / Les liposarcomes rétropéritonéaux. A propos de deux cas.

We report two cases of retroperitoneal liposarcoma that required multiple surgical excisions for locoregional relapse diagnosed by surveillance based on CT-scan. One patient has had adjuvant radiotherapy. With the literature review, we discuss the pathologic and therapeutic aspects of these lesions.

[Cysts of the prostate. Apropos of 2 cases]. / Les kystes de la prostate. A propos de deux cas.

Two prostatic cysts have been diagnosed by us since 1993. The first patient presented with dysurial the second cyst was discovered incidentally. Transrectal ultrasound examination was useful for diagnosis. Treatment was a puncture--aspiration of the cyst in the first case, and abstention in the second one. A review, of the epidemiological, pathological and therapeutic aspects of the prostatic cysts is presented.

[Colonic polyps at the level of the site of an ureterosigmoidostomy 11 years after conversion of a Coffey operation to a continent ileocecal bladder. A case]. / Polypes du côlon au niveau du site d'une urétérosigmoïdostomie 11 ans après la transformation d'un Coffey en une vessie iléocaecale continente. Un cas.

The case of precancerous polyp arising at the two sites of ureterosigmoidostomy 11 years after conversion to continent ileocaecal conduit is reported. The authors discuss risk factors, presenting conditions, therapeutic implications and preventive measures.

[Metanephric adenoma: an unusual tumor of the kidney. Apropos of a case]. / L'adénome métanéphrique: une tumeur rare du rein. A propos d'un cas.

The authors report an uncommon case of renal adenoma, a metanephric adenoma in a 21 year-old man. Treatment consisted in a tumorectomy. The review of the literature, shows it to be a benign tumor with no malignant potential.

[Results of endorectal MRI in local staging of prostatic cancer. Correlation with specimens from prostatectomy. Apropos of 47 cases]. / Résultats de l'IRM endo-rectale dans la stadification locale du cancer de la prostate. Corrélation avec les pièces de prostatectomie. A propos de 47 cas.

Endorectal surface coil magnetic resonance imaging (MRI) was used in the local staging of prostate cancer in 47 patients. We used an 1.5 Tesla General Electric magnet. Fast spin echo sequences were acquired in all cases. All pathological specimen were reviewed by one pathologist. Pathological study showed that 19 patients had a locally confined cancer of the prostate (pT2), and 28 had an extraglandular extension (pT3). MRI correctly predicted a pT3 tumor in 15 of 28 cases, and a pT2 tumor in 18 of 19 patients. MRI was 70% accurate in the differenciation of stage pT2 from stage pT3 cancer. One case was overestimated and 13 cases were underestimated. The latter 13 patients had microscopic extracapsular invasion only.

[Malignant adrenocortical tumors. Apropos of 9 cases]. / Cortico-surrénalomes malins. A propos de neuf cas.

The authors report 9 cases of adrenocortical carcinoma: 6 women and 3 men. 5 cases (55%) had a "nonfunctioning" tumor and 4 cases (45%) presented a clinical endocrinologic syndrome. According to the MacFarlane classification, patients were classified as: stage I: 1 case, stage III: 6 cases and stage IV: 2 patients. Adrenalectomy was performed in 7 cases. There was one postoperative death. 5 patients obtained clinical improvement.

[Herald's lesion with a localization in the bladder. Apropos of a case]. / La lésion de Hérald à localisation vésicale. Apropoa d'un cas.

Herald's lesion located in the bladder is an non-specific extrinsic inflammatory lesion. This pseudotumoral neoformation often results from a pathologic process of the pelvis (95%). Recurrent cystitis is the main symptom. The diagnosis is difficult and cystoscopy and biopsy is the basic investigation. Surgical treatment must remove the original lesion with partial or total cystectomy. Epidemiologic, pathologic and therapeutic aspects of this disease are discussed.

[A rare variety of urothelial carcinoma. Apropos of 2 cases]. / Une variété rare de carcinome urothélial. A propos de 2 cas.

We report two cases of grade 1 papillary transitional cell carcinoma which have a distinct pathological. The tumor was infiltrating the lamina propria in the first case (pT1) and the superficial muscle in the second case (pT2). These tumors were composed by nests and tubules with few cytological atypias, making the diagnosis of cancer difficult. The prognosis of these tumors is not well defined.

[Severe algoneurodystrophy of the right foot associated with prostatic cancer]. / Algoneurodystrophie sévère du pied droit associée à un cancer prostatique.

The authors report 1 case of particularly severe reflex neurovascular dystrophy whose clinical course was marked by the discovery of a carcinoma of the prostate. There was improvement in the reflex neurovascular dystrophy despite hormonal therapy of the cancer. Reflex neurovascular dystrophy cannot be considered as a form of a paraneoplastic syndrome.

[Renal oncocytoma. Apropos of a new case. Diagnostic and therapeutic problems]. / Oncocytome rénal. A propos d'un nouveau cas. Problème diagnostic et thérapeutique.

The authors report a case of renal oncocytoma. Ultrasonographic and C.T. imaging has considerably simplified a preoperative diagnosis of this rare renal tumor; true diagnosis was only confirmed by histological examination. Surgical treatment counted in radical nephrectomy.

[Xanthogranulomatous pyelonephritis. Apropos of 11 cases]. / Les pyélonéphrites xanthogranulomateuses. A propos de 11 cas.

Xanthogranulomatous pyelonephritis is a rare manifestation of chronic pyelonephritis. 11 cases were treated between 1988 and 1993. Patients age ranged from 17 to 60 years (mean 34 j). A pseudotumoral form was documented in 3 patients and pyonephrosis was found in 8 cases. Urine culture was usually positive for a non specific germ. Imaging technics could differentiate pseudotumoral disease from diffuse disease. Treatment consisted in a nephrectomy.