[Extra-nodal NK/T-cell lymphoma, nasal-type, revealed by cutaneous and ocular involvement]. / Atteintes cutanée et oculaire révélatrices d'un lymphome T/NK extra-nodal de type nasal.

BACKGROUND: Extra-nodal NK/T-cell lymphoma (ENKTL) is a form of highly malignant non-Hodgkin's lymphoma. There are two types: nasal forms primarily affecting the oropharyngeal sphere and so-called nasal-type extra-nasal forms in which primary skin involvement is the most common feature enabling diagnosis. Herein, we report a case of systemic nasal-type ENKTL (ENKTL-NT) that was diagnosed based on skin involvement associated with ocular involvement. PATIENTS AND METHODS: A 67-year-old female patient, without immunodepression, was admitted to the dermatology department for a worsening inflammatory scaly patch of skin on her right calf. Secondarily, further lesions appeared on her body as well as a generalized macropapular rash and sores. These were associated with fever spikes, as well as ophthalmoplegia and edema, preventing her from opening her right eyelid. Tests for infectious, autoimmune and inflammatory disorders were negative. A cerebro-orbital scan revealed infiltration and contrast enhancement of the right periocular fat without any mass effect or cerebral extension. A positron emission tomography (PET) scan revealed multiple hypermetabolic skin lesions. Histological analyses indicated dermal-hypodermal lymphomatous tumor proliferation, and immunohistochemical analyses revealed lymphocytes expressing NK-cell markers (strong CD56+ expression), cytotoxic markers (granzyme B and TIA-1), and the presence of Epstein Barr virus (EBV) in the tumor cells. The patient was diagnosed with systemic ENKTL-NT. Her condition deteriorated rapidly, with the onset of refractory macrophage activation syndrome leading to death due to multiple organ failure. DISCUSSION: Skin involvement in ENKTL is non-specific and uncommon, which can delay diagnosis. Treatment is based on polychemotherapy comprising L-asparaginase and possibly consolidation therapy with autologous or allogeneic hematopoietic stem cell transplantation. The prognosis of ENKTL-NT is poor due the more aggressive nature of the disease compared with the nasal forms, with frequent visceral involvement and macrophage activation syndrome. Skin involvement seems to be a poor prognostic factor. Although ocular involvement is documented, its association with skin involvement is rare and mainly secondary to nasal forms of ENKTL. This case of an extra-nasal form of ENKTL-NT with systemic involvement illustrates the difficulty of diagnosis and the poor prognosis of this type of lymphoma.

[Extensive digital necrosis during dermatomyositis associated with MDA-5 antibodies]. / Nécroses digitales étendues au cours d'une dermatomyosite avec anticorps anti-MDA-5.

BACKGROUND: Dermatomyositis (DM) is an inflammatory disease associated with auto-antibodies in 50 to 70% of cases. A new antibody, anti MDA-5, has been described in association with a specific type of DM involving severe interstitial lung disease and minimal muscle disease. We report the first case of DM with MDA-5 antibodies and with interstitial lung disease and rapidly extensive digital necrosis. PATIENTS AND METHODS: A 28-year-old male was hospitalized for asthenia, myalgia and subacute dyspnea. Examination demonstrated skin lesions with edema on every digit associated with purpuric and cyanotic lesions, as well as erythematous papules on the helix and the elbows, and Gottron's papules. Systemic corticosteroid therapy was initiated. The immunoprecipitation results indicated the presence of anti-MDA-5 antibodies. Despite corticosteroid therapy, the patient's respiratory status gradually deteriorated towards pulmonary fibrosis and rapidly extensive necrosis appeared on all fingers and toes. Theses effects were resistant to cyclophosphamide and immunoglobulin but were stabilized by cyclosporine. DISCUSSION: Anti-MDA-5 antibodies are specific to DM and constitute a risk factor for severe interstitial lung disease (70% of cases) with a higher risk of mortality (40%). The cutaneous presentation of this DM is specific with palmar papules and mucocutaneous ulceration. Rapidly extensive digital necrosis has not been previously reported. No treatment has demonstrated superiority. CONCLUSION: We report the first case of DM with anti-MDA-5 antibodies involving interstitial lung disease and massive digital necrosis. Because of the pulmonary risk, in the presence of clinical lesions containing anti-MDA-5 DM, screening for these antibodies should be carried out.

[History of surgery of the parathyroid glands]. / Histoire de la chirurgie des glandes parathyroïdes.

In 1850 Sir Richard Owen discovered the parathyroid glands in rhinoceros. After they have been discovered in man in 1880 their history spread all along the 20th century. The history started from the descriptive anatomy and the clinical pictures of their illness to the trouble of their functioning. The hormone was studied while the surgeons began to cure adenoma, hyperplasia and cancers.

[Lymph nodes and rectal cancer]. / Ganglions et cancer du rectum.

Nodal invasion is a major prognostic factor of rectal cancer. Lymphatic extension of rectal cancer usually involves the mesorectal nodes then the inferior mesenteric chain but in 14% of patients, particularly with cancer of the lower rectum, metastasic nodes can be observed in the internal or lumboaortic chains situated beyond the usual territory of nodal dissection. On average, 30 nodes are examined on a proctectomy specimen, but with wide interindividual variation. The tumor can be adequately staged if at least 15 nodes are examined with little risk of not recognizing nodal metastasis. Metastatic nodes of rectal cancer are almost always small, more than 90% measuring less than 10 mm and 70% less than 5 mm. The number of invaded nodes and the total number of examined nodes are prognostic factors for survival. Hypofrationated preoperative radiotherapy does not alter the nodal status but a long radiotherapy protocol (45 Gy over 5 weeks) reduces significantly the total number of nodes examined without changing the number of metastasic nodes. Micrometastases (measuring less than 2 mm), identified by immunohistochemistry or gene amplification, can be detected in 25 to 70% of nodes considered metastasis-free at the usual microscopic examination. The prognostic value of these micrometastases remains to be established. The first node draining the tumor (sentinel node), which can be detected rapidly with dye infusion, appears to provide a good picture of the nodal status, the risk of finding an invaded node if the sentinel node is metastasis-free is less than 5%.

[Role of surgery in closed abdominal trauma]. / Place de la chirurgie dans les traumatismes fermés de l'abdomen.

Over the past twenty years, nonoperative management has increasingly been recommended for the care of patients with blunt abdominal trauma. Emergency laparotomy remains the rule in patients with hemodynamic instability or in those with peritonitis due to intestinal perforation. Surgical treatment of liver and splenic lesions tends to be more conservative. After assessment of the lesions by computed tomography, nonoperative management in intensive care unit is allowed in the majority of patients.

Extracorporeal lithotripsy of ureteric calculi using the Dornier HM-3 lithotriptor.

Over a 5-year period (November 1984-November 1989), we treated 356 patients with ureteric calculi; 170 were treated by extracorporeal shock wave lithotripsy (ESWL) on a Dornier HM-3 lithotriptor. The calculi (n = 176) were uniformly distributed along the length of the ureter: 44 were just below the pelviureteric junction, 59 were lumbo-iliac, 42 were in the upper bony pelvis and 32 in the lower bony pelvis. The mean diameter of the upper ureteric calculi was 10 mm and for the others it was 8 mm. Thirty-four patients with acute obstructive pyelonephritis required pre-ESWL drainage of the urine. X-ray localisation required intravenous urography during lithotripsy in 52 cases (30%). On plain X-ray the following day 170 stones (96%) were judged to have disintegrated. The 6 patients whose stones were not fragmented received further treatment (ureterotomy (4) and ureteroscopy (2)). Five patients required additional treatment because of pain or fever (catheterisation (3) and ureterotomy (2)) and 2 patients had a second lithotripsy owing to insufficient fragmentation. Four patients were lost to follow-up. In 153 patients (90%) the fragments were eliminated completely, 146 in the first month and the remainder before the sixth month. No serious sequelae were observed. In addition to the 5 patients who required supplementary treatment. 11 patients with pain or fever needed medical treatment. We recommend first intention in situ ESWL for all ureteric calculi.

Tumors of the upper urinary tract: 10 years of experience.

We reviewed 108 patients with upper urinary tract tumors who underwent surgical treatment during a 10-year period (87 men and 21 women with a mean age of 63.5 years). Of the tumors 97% were unilateral and only 3 patients had bilateral tumors. Two-thirds of the patients had a single tumor focus and a third had 2 or more tumor foci. Additionally, there were 31 patients (28.7%) with previous and/or simultaneous bladder tumors. Nephroureterectomy was performed in 92 cases, nephrectomy in 6 and a conservative operation in 13. In 65 cases lymphadenectomy was added. The survival rates at 5 and 10 years were 67 and 65%, respectively. Of the patients 90% with cancer-related deaths had high grade tumors. Of the 15 patients with positive lymph nodes 87% died of metastasis compared to 8% of the 50 patients with negative lymph nodes. Nine patients (8.7%) had relapse in the upper urinary tract, 6 (5.8%) in the ipsilateral ureter and 3 (2.9%) in the contralateral ureter. Of these 3 patients 2 had recurrent multifocal bladder tumors. For patients who present with an upper urinary tract tumor the risk of a bladder cancer was approximately 9% and that of a contralateral urothelial tumor was 1%.

[Do posterior urethral valves alter the results of renal transplantation in children?]. / Les valves de l'urèthre postérieur altèrent-elles les résultats de la transplantation rénale chez l'enfant?

The authors conducted a retrospective study based on a series of 715 renal transplantations in children performed between January 1973 and December 1989 at the Hôpital Necker-Enfants Malades in order to determine whether the long-term result of renal transplantation was as good in children with posterior urethral valves (PUV) as in children with a normal lower urinary tract. Group 1 consisted of 50 renal transplantations performed in 41 children in whom the primary urological disease was PUV. The bladder was able to be used without modification in 36 cases and had to be enlarged in 5 cases. A permanent cutaneous urinary diversion was not required in any of these transplanted patients. Group 2 consisted of 665 renal transplantations performed in children without PUV. There was no significant difference between the two groups in terms of the distribution of cadaver kidney and living related donor transplantations. Immunosuppressive treatment consisted of various combinations of prednisone, azathioprine, ALS for the earlier patients in the series, OKT 3 and cyclosporin for the more recent patients. In group 1, we observed a urological complication rate of 20% and a vascular complication rate of 14%, while, in group 2, the urological complication rate was 16.9% and the vascular complication rate was 9.5%. The actuarial 5-year and 10-year graft survival rates were 63% and 49% in group 1 and 63% and 49% in group 2, respectively. The actuarial graft survival rates were therefore identical in the two groups.(ABSTRACT TRUNCATED AT 250 WORDS)

Expression of the human nephron differentiation molecules in renal cell carcinomas.

The authors tested frozen sections from 28 renal cell carcinomas (RCC)--21 clear, 1 eosinophilic, 4 basophilic, and 2 spindle-shaped cell type--with monoclonal antibodies (MAb) reacting against cytokeratin, vimentin, CD24, CALLA/CD10, villin, CD26, and HLA class I and class II molecules. These molecules are markers of specific segments of the mature kidney, and their loss or acquisition reflects the different steps of human nephrogenesis. KI67 MAb was used to evaluate cell-proliferating activity. All RCC cases expressed cytokeratin. Coexpression of vimentin was observed in 21 of 28 cases. Whether of clear or chromophilic type, all tumoral cells strongly expressed CD24 molecule, present on primitive blastema cells. All clear-type RCCs expressed CALLA/CD10 and 60% were also villin positive; some were faintly positive for CD26. CALLA, villin, and CD26 were not detected in basophilic cell type. HLA class I molecules were variably expressed in almost all cases, but HLA class II were never detected on tumoral cells. Except for the spindle-shaped population, cell-proliferating activity was low. These results favor the hypothesis that RCCs derive from cells that have 'recovered' the different options of metanephric differentiation. Clear cells show evidence of maturation toward proximal type, while basophilic cells do not. It would be of interest to evaluate the usefulness of serum measurements of villin and/or CALLA as markers in clear cell-type RCC.

[Relationships between posterior urethral valves, vesico-renal reflux and renal dysplasia]. / Les liens entre valves de l'urèthre postérieur, reflux vésico-rénal et dysplasie rénale.

Children with valves of the posterior urethra show asymmetric involvement of the upper urinary system with a nonfunctional kidney in 10 to 15% of cases. In a study of 12 nephrectomy patients and 14 reflux patients, the authors reached the conclusion that valvular obstruction, vesico-renal reflux and dysplasia are coexisting anomalies with little relationship between each other.

[5-year treatment results of urethral stenosis using internal urethrotomy in adults]. / Résultats à cinq ans du traitement des sténoses de l'urètre par urétrotomie interne chez l'adulte.

The authors reviewed the records of 69 patients with a mean age of 51 years treated for single (81%) or multiple (19%) urethral strictures, in whom the first operation to be performed was endoscopic internal urethrotomy with a follow-up of at least 5 years. The mean follow-up was 71 months. The actuarial rate of good results was 27.2 +/- 11% at 5 years. The success rate of repeated internal urethrotomy was satisfactory, but was not evaluated with the same follow-up (62.5% after the first repeat urethrotomy with a mean follow-up of 13 months).

[Superficial bladder tumors. Results of preventive treatment of recurrence by intravesical instillation of BCG]. / Tumeurs vésicales superficielles. Résultats du traitement préventif des récidives par instillations endovésicales de BCG.

Two-thirds of newly diagnosed bladder tumors are superficial transitional cell carcinomas. Local relapses occur in more than 2/3 of the patients after transurethral resection of the initial tumor, with a risk of muscle invasion in 1/3 of them. A retrospective study of 100 consecutive patients was performed to assess the role of intravesical BCG-therapy as a prophylaxis against the recurrence of superficial bladder cancer; the treatment protocol is described. Eighty-two patients had previously had recurrent tumors and 18 were given BCG directly because they had multiple, large, high grade or widespread tumors. Seventy patients (70 p. 100) tolerated the treatment well, but 6 experienced serious complications (3 systemic BCG infections and 3 bladder contractures). Of the 18 patients given BCG after their first tumor, 12 (66 p. 100) are tumor-free with a mean follow-up of 13 months. Of the 82 patients with previous recurrent tumors, 52 (63 p. 100) were improved by the treatment. Of the 100 patients, twelve tumors became muscle invasive and required more aggressive intervention. Intravesical BCG is effective in preventing recurrences of superficial bladder cancer. However, the side effects and the risk of muscle invasion require close monitoring.

[Tumors of the upper excretory urinary tract. A homogeneous series of 108 patients]. / Tumeurs de la voie excrétrice urinaire haute. Une série homogène de 108 patients.

In their study of a homogeneous series of 108 patients operated for a transitional cell tumor of the upper urinary tract, the authors emphasize the following points: --every third tumor has several foci, and it is very difficult to make a complete inventory of all tumoral foci before surgery; --the great majority of these tumors are unilateral. Bilateral recurrence following radical unilateral excision is quite exceptional; --low-grade superficial tumors virtually never invade the lymph nodes, and their prognosis is excellent as a rule; --grade III carcinomas area almost always infiltrating and present high risks of lymph node invasion. Lymph node involvement means that the evolution will be lethal in all cases. None of the patients having invaded lymph nodes survived; --metastases appear soon, usually within the first 24 months, but mainly between the 6th and 12th months following surgery; --20% of the patients in this series died of metastases; --the actuarial survival rate after 5 years is 68%.

[Extracorporeal shockwave lithotripsy in children]. / La litotricia extracorpórea por ondas de choque en el niño.

Upper urinary tract lithiasis is a rare condition in children in comparison to its incidence in the adult population. The distribution of lithiasis in children has been reported to be 56% for idiopathic lithiasis, 20% for metabolic lithiasis, and 25% for lithiasis associated with uropathy. Extracorporeal shock wave lithotripsy (ESWL) has become the treatment of choice for upper urinary tract calculi in the adult patient as well as in children after minor changes and complementary material had permitted the utilization of the Dornier HM-3 lithotripter in these small patients. The reported 3-month success rates range from 47% to 83%, according to the number of patients or kidneys free from calculi. The series reported in the literature have a mean of about 60% completely stone-free kidneys evidenced on the X-ray of the abdomen without preparation at 3 months. Its indications and results are comparable with those of ESWL in the adult. However, the long-term results have as yet not been evaluated and, to date, its hypothetical effects on renal growth or arterial pressure have not yet been elucidated.

[Anomaly of the pyelo-ureteral junction and antenatal ultrasonic diagnosis]. / Anomalie de jonction pyélo-urétérale et échographie anté-natale.

Over a period of five years, 40 cases of anomaly of the pyelo-ureteral junction were diagnosed ante-natally which subsequently required surgical intervention during the first year of life. The indication for the therapy was based on the degree of obstruction (IVU, DTPA scanning, Whitaker test), the thickness of the renal parenchyma (IVU, ultrasound) and, to a lesser extent, on calyceal dilatation. After a period of follow up ranging from 3 to 48 months (mean 16.8 months), good results were noted in 37 cases, there were two permeable junctions with poorly functioning kidneys and one obstruction which was successfully reoperated. These results suggest that intervention is preferable to surveillance at varying intervals for an undetermined period, where the risk of a sudden exacerbation is always possible.

[Value of extracorporeal shockwave lithotripsy in the treatment of urinary lithiasis in children]. / Intérêt de la lithotriptie extra-corporelle par ondes de choc pour le traitement de la lithiase urinaire de l'enfant.

We report our experience with extracorporeal lithotripsy (Dornier HM3) in a series of 26 children with a mean age of 11.6 years, treated for 3 1/2 years. 12 children (46%) had a previous history of calculi and 7 (27%) had already undergone surgery on the same side. Treatment requires two transducer investigations before lithotripsy. With the exception of minor modifications the technique is the same as in the adult. Three (9.7%) postlithotripsy complications were noted, requiring 2 drainage procedures to be carried out on the urinary tract. At 3 months, the success rate (no residual calculi on the plain abdominal film) was 60.7%. This study confirms the efficacy of lithotripsy in the treatment of urinary lithiasis in children.