Dermal malignant melanoma developing from a nevus of Ota.
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Biomedical subjects
Publications and source records attributed to J C Mandard.
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A 72-year-old woman presented with rapidly progressive renal failure and multiple myeloma. The patient died 6 months later of severe hepatic insufficiency. The light-microscopic, immunological and ultrastructural findings showed widespread kappa-light-chain deposits including the kidneys, liver, spleen, heart, lungs, tongue, ovary, pancreas and bone marrow associated with massive AL amyloid deposits in the same organs and in the thyroid gland. The concurrent presence of two different deposits is very unusual and the possible mechanisms for such an association are discussed.
The authors report 2 cases of malignant eccrine poroma (M.E.P.) located on the face: one on the auricle which was treated by triangular shape resection, the other on the nasal tip which underwent a reconstruction with a Converse scalping flap after large surgical resection removed underlying skin and cartilages. A study reviewing the main features of M.E.P. is presented on the basis of these clinical cases. M.E.P. are skin appendage tumors originating from the intra epithelial portion of the eccrine sweat duct. Such denominations as malignant hidroancanthoma, eccrine porocarcinoma, poroepithelioma, correspond to the same tumor. They are very rare: 73 cases have been reported in the world literature with only 12 located on the face; the main localisations involve the lower extremities, but the palmo plantar areas are involved more frequently in benign tumors. Elderly people over 60 are concerned in almost all cases. Many of the patients have noticed a lesion for years before a diagnosis was made suggesting one of the etiopathogenic theories that M.E.P. may result from malignant transformation of a benign eccrine poroma. Their clinical appearance is non specific nevertheless their malignant nature is suspected and misdiagnosis with basal cell carcinoma is frequent. Microscopic findings demonstrate the diagnosis by showing the association of malignant features with eccrine poroma. These tumours invade both by superficial spead and by deep extension towards dermal and hypodermal structures. Their prognosis is marked by the rapid development, in approximately on half of cases, of cutaneous, lymp node and visceral metastases. Clinical and histological examination have no prognostic value exception the case of deep invasion.(ABSTRACT TRUNCATED AT 250 WORDS)
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Pericardial abnormalities remain the most common manifestation of radiation-induced cardiac disease, but coronary artery lesions are not rare. In this report we describe a left coronary ostial stenosis which appeared five years after mediastinal irradiation for breast carcinoma in a 50-year-old woman. The patient underwent coronary angiography. A pressure drop was observed as the left catheter tip engaged the left coronary ostium; so, only nonselective coronary opacifications were performed showing an isolated, marked narrowing of the left coronary ostia. During surgery, a circumferential aortotomy allowed the examination of the left coronary ostium which appeared severely stenosed. The coronary tree was otherwise normal. A termino-terminal saphenous vein graft was anastomosed on the left stem and its proximal part was implanted on the ascending aorta. The coronary ostium and the proximal part of the left main stem were excised and the macroscopic examination of the proximal part of the left coronary artery confirmed the diagnosis of severe ostial stenosis. Microscopic examination of the coronary ostium showed a severe intimal thickening without any evident lesion of the media. This intimal thickening consisted of fibrous tissue without extracellular lipid deposit. Microscopic examination of the aorta near the coronary ostium also demonstrated an intimal thickening without any lesion of the media. Coronary ostial stenosis appears to be a rare lesion; its incidence has varied between 0.13 and 2.7% in angiographic studies and there is co-existing disease in multiple coronary vessels in the majority of cases.(ABSTRACT TRUNCATED AT 250 WORDS)
We report for the first time a case of Crohn's enteritis associated with a chronic tubulo-interstitial nephritis in an adolescent. The illness started insidiously in an 11 year-old boy who had suffered from failure to thrive and protracted watery diarrhea. At presentation the patient had an inflammatory bowel disease located to the left colon but no renal dysfunction. Until the age of 15, the intestinal symptoms were stable but a progressive renal insufficiency developed. A percutaneous renal biopsy was then performed which showed a widespread chronic tubulo-interstitial nephritis. Following a 6 months corticosteroid treatment, renal dysfunction seemed to be stabilized. However, delayed growth and corticosteroid dependency led to total colectomy. Pathologic examination showed granulomatosis involvement of the entire colon and severe interstitial nephritis with neither linear nor granulous deposits along tubular basement membranes. After a 2 year-delay following the colectomy, renal function was stabilized and a catch up growth was achieved.
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We report 5 cases of isolated ostial stenosis of the left main coronary vessel. Isolated ostial stenosis occurs preferentially in young or middle aged women for whom coronary insufficiency is usually not a serious threat. Patients with this type of lesion have characteristically severe angina of relatively recent onset. The condition may be difficult to diagnose at angiography, but a fall in pressure when the tip of the catheter enters the coronary lumen beyond the stenosis, a lack of reflux of the contrast medium into the sinus of Valsalva during intracoronary injection and its persistence in the coronary vessel should alert the investigator. A pathological study of 3 cases revealed typical atheromatous lesions in 2 patients (with extension of an aortic plaque to the left coronary ostium in one, and atheroma localized on the ostium in the other) and a purely fibrous lesions in a patient who had undergone thoracic radiotherapy 5 years previously. Although relatively rare, stenosis must be diagnosed in view of its sombre spontaneous prognosis (one patient died 3 days after coronary arteriography), of the risk of underestimating its frequency, and of the hazards of selective coronary catheterization in such patients (one of our patients died 15 minutes after coronary exploration).
10 cases of keratoderma climactericum are reported. This keratosis of the palms and soles appears late in women of menopausal age. The keratotic lesions first develop at the plantar pressure points, making walking troublesome. Involvement of the hands remains discrete. Examination for contact allergy, fungal tests, vitamin A serum levels, and sex hormones were negative or normal in all the 10 patients. Microscopy revealed a lichenified eczema with evidence of mechanical irritation. Etretinate (0.78 mg/kg/day) brought about partial or total remission of the hyperkeratosis. Pain on walking disappeared in all the patients.
The authors report a new case of luteinized hyperplastic cystic ovary in a pregnancy that was in every other respect normal. This syndrome of ovarian hyperstimulation is most often found in patients suffering from trophoblastic diseases or following iatrogenic accidents. It is exceptionally rare in a non-molar pregnancy. The comparison with the other form of ovarian hyperactivity that gives rise to a luteoma of pregnancy emphasizes the questions that have to be answered of the hormonal mechanism leading to maternal and fetal virilisation that complicate these two pseudo-tumours and how they are handed on from one generation to the other, and what the mechanisms is of placental HCG in this abnormal condition.
On the occasion of a case of Di George syndrome, complicated by a post-transfusion lethal graft versus host reaction, the authors review its main clinical and biologic criteria. This case also shows that immune deficiencies should be diagnosed early in order to adopt preventive measures before transferring the patient in a specialized unit, where immunorestitution will be discussed.
One hundred surgical specimens from patients with esophageal cancers were studied in detail. The characteristics of the cancers were similar to those described in the literature. Subserial sectioning of the specimens permitted determination of the type, number, and extent of noncancerous mucosal lesions and their exact location in relation to the carcinomas. Ninety-five per cent of the resected esophagi contained at least one focus of intraepithelial neoplasia, for the most part adjacent to the invasive carcinoma; in 14 per cent of the cases, intraepithelial carcinomas were detected at some distance from the invasive carcinomas. The invasive carcinomas involved an average of 20 per cent of the mucosal surface and the intraepithelial neoplasias, 5 per cent. The noncancerous mucosal lesions were analyzed, and their extent was evaluated. Esophagitis, parakeratosis, atrophy, dyskeratosis of the epithelium, and hyperplasia and metaplasia of the mucosal gland ducts were encountered. The exact significance of these lesions in esophageal carcinogenesis is not known. The mucosal abnormalities associated with preoperative irradiation are defined. The results of iodine and toluidine blue testing are presented.
Primary hyperparathyroidism and sarcoidosis were found in one patient with hypercalcemia and renal failure. Cervicotomy disclosed a parathyroid adenoma and renal biopsy demonstrated interstitial nephropathy. Association of sarcoidosis with hyperparathyroidism, if not fortuitous, has not yet been clearly established.
Hyperparathyroidism secondary to chronic renal failure is often accompanied by metastatic calcification with involvement of the skin. The cutaneous lesions appear to be of two types: in the first there was massive calcification of the skin and the subcutaneous tissue; in the second there was necrotic purpura due to intimal proliferation and medial calcification of the cutaneous arterioles. We report the case of a 75-year-old-woman with osteodystrophy who had painful necrotic ulcer and reticulated purpura of the legs (fig. 1 a, b). The new lesions progressed by lateral extension and central necrosis. Biopsy of the skin underlying the purpuric areas revealed intimal proliferation with narrowing of the lumen (fig. 2 a, 3). The most prominent feature was the considerable intimal and internal elastic membrane calcification in several small arteries (fig. 4 a, 5). Von Kossa's stain showed calcium to be present (fig. 2 b, 4 b). There was no calcification of the tunica media. Serum calcium phosphorus product was increased (table I). Tubular reabsorption of phosphate was abnormally low. The serum immunoreactive parathyroid hormone was normal. An iliac crest biopsy revealed the classical lesions of osteodystrophy. Her skin lesions showed spontaneous resolution without parathyroidectomy: the ulcers were healing within two months. A progressive reticulated necrotic purpura has previously been reported in chronic renal failure with secondary hyperparathyroidism. These black ischemic lesions bear a strong resemblance to those accompany atheromatous necrotic angiodermatitis. The metastatic calcification seen in uremia often involves the media; the present case is the first to be reported with intimal calcification.(ABSTRACT TRUNCATED AT 250 WORDS)
Malakoplakia is a rare granulomatous lesion involving rarely organs other than the urinary bladder. 39 cases of colo-rectal Malakoplakia have been published. 12 of them are associated with a colo-rectal tumor. The authors report in this paper a new case of rectal Malakoplakia associated with an adenocarcinoma. An ultrastructural study show septate junctions between the phagolysosomes. This very rare feature and the physiopathology of the association Malakoplakia-adenocarcinoma are discussed.
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