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The authors report here two observations of leiomyosarcomas arising from peripheral blood vessels. The first case occurred in the pedious vein and infiltrated into tarsal bones. The second case was localized into leg muscles' tendons, near the knee. The topographical and histological characteristics of peripheral vascular leiomyosarcomas are noted, and compared, according to the review of literature, with those of the great central blood vessels, and of other classical leiomyosarcomas.

An experiment in adult rats with histologic study of isolated bone segment revascularization is reported vascular bundle is inserted into the humeral head that has been transferred under abdominal skin. It induces an active proliferation of new blood vessels with formation of new bone/invading the necrotized bone.

Cowden's disease, also called multiple hamartoma syndrome, is a clinical entity characterized by hamartomatous tumours of endodermal, mesodermal and ectodermal origin. Although extremely rare, the disease must be known to all internists. A case of Cowden's disease in a 36-year old male patient is reported. The authors insist on the high incidence of digestive disorders and the risk of malignant degeneration of mammary and thyroid tumours. They also describe the cutaneous and mucosal lesions characteristic of the disease.

The prognostic value of bone marrow biopsy was established in 31 patients suffering from stage IIA and IIIA myeloma (Durie and Salmon classification). Data obtained from quantitative marrow cytology permit to differentiate 3 sub-groups of cells: plasmocytes, plasmoblasts, plasmocytes/plasmoblasts of which the incidence on the prognosis and the response to treatment was studied. There is a correlation between the response to MP and the nature of various cells populations and, on the other hand, the type of infiltration. A sub-group of patients, resisting to MP, was identified: diffuse plasmoblastic myelomas of poor prognosis. Induction poly-chemotherapy seems far superior over the MP combination, which was always a controverted issue.

Sacroiliac joint (SIJ) involvement has been reported in up to 9.7 percent of patients with skeletal tuberculosis. Lack of awareness of this now uncommon form of infection often leads to diagnostic delay and increased morbidity. Eleven consecutive cases of SIJ tuberculosis are reported; clinical and radiologic features, diagnosis, treatment, and outcome are discussed. Buttock pain was the presenting complaint in all patients. However, radicular pain in the lower back (seven patients) or lower limb (10 patients) was common and in one patient precipitated an unnecessary surgical intervention. SIJ tuberculosis is frequently an isolated phenomenon. Therefore, direct sampling of the SIJ is necessary to establish the diagnosis. The recently described technique of closed needle biopsy of the SIJ was employed in all 11 patients and established the diagnosis in nine of the 11.

The results of 20 puncture-biopsies and 43 needle-biopsies performed under local anaesthesia and guided towards a localized bone lesion under a fluoroscopic TV screen are reported. Malignancy was confirmed in 31 out of 36 cancerous bone lesions (83%). Metastases were demonstrated in 24/25 cases (96%) and malignant lymphocytic or plasmocytic lesions in 6/6 cases. There were no false-positive results with benign lesions. However, an accurate aetiological diagnosis was supplied by puncture- or needle-biopsy in only 1/5 cases of primary malignant tumour and in 1/6 cases of benign tumour. Guided puncture- or needle-biopsy seems to be particularly useful to distinguish between benign and malignant lesions, notably when bone metastasis is suspected. The technique can then be applied to those cases where puncture-biopsy of the posterior iliac crest is negative or even as first choice procedure if the bone lesion is not readily accessible.

Diffuse inflammatory angiitis is seen in extremely varied aetiological and nosological situations. In some cases, the vascular disease represents the only sign of systemic disease. In other cases, it mays be associated with or complicated by diffuse systemic inflammatory conditions. This study analysed 100 cases of inflammatory angiitis with systemic manifestations seen over a period of 7 years in a rheumatology department. Patients with scleroderma, erythema nodosa, Behçet's syndrome and angiitis associated with microbial infectious diseases were excluded from this study. 24 cases in which the angiitis was clinically certain, but in which the vascular lesion was not confirmed histologically were also excluded. The type of histological lesion and the nosological distribution of these 100 cases are described in detail. The results of this analysis confirm the very great variety of the clinical and histological presentations. A number of rare syndromes were observed. Classical periarteritis nodosa only represented 14 per cent of the whole series and only 3 of the 14 cases had a demonstrable aetiology (2 HBs, 1: desensitisation to Candidin). The authors stress the histological diversity of inflammatory angiitis associated with rheumatoid arthritis and systemic lupus erythematosus. All sizes of vessels may be involved and all types of lesions can be observed in these two diseases. The authors also emphasise the lesser known clinical and histological features (stenosing endarteritis of the great vessels in systemic lupus erythematosus) and previously unreported cases (valvular lesions in 2 cases of Mac Duffie's syndrome, progression of 2 cases of delayed urticaria to pressure towards a clinical lupus with unusual laboratory findings).(ABSTRACT TRUNCATED AT 250 WORDS)

The authors report 17 cases of primary tumors of the knee treated by wide resection, removing "en bloc" the entire epiphysis in 14 cases and in the remaining 3 most of the epiphysis. The series consisted of 8 giant cell tumors of which 6 were followed for between three and fifteen years, and for 9 tumors of moderate malignancy amongst which 1 fibrosarcoma, 1 parosteal osteosarcoma and 3 cartilaginous tumors which were followed between five and fourteen years. All were free of metastatic spread except 1 fibrosarcoma whose resection was debatable. Arthrodesis was carried out on all but 2 patients received prosthesis. The arthrodeses were made by autogenous grafts protected in 11 cases by intramedullary rod, in 1 case by a plate, and in 3 cases by both. The series contains one amputation and three fractures necessitated secondary grafting with replacement of rod in two cases. The results are analysed in carcinological and technical shemes. The value of resection and the importance of grafting over the arthrodesis are emphasised. In the long term, resection-arthrodesis means the conservation of a stable and functional limb, permitting an active life one year postoperatively. Massive prosthesis are only exceptionally indicated.

We report three cases of inflammatory fibroid polyp of the stomach (Helwig's pseudo-tumour) and record 127 patients in the literature. The anatomo-clinical features of such a disease are analysed and the larger outline of eosinophilic infiltrations of the gastro intestinal tract are described. Most of the authors class the gastric nodular eosinophilic granuloma as the Helwig's pseudo-tumour, but they are not so unanimous for the exact relationship between inflammatory fibroid polyp and the eosinophilic gastroenteritis. For Helwig, they are two distinct conditions, but for others, they belong to the same anatomo-clinical entity, with a broad diversity of morphological features.

Pregnancy concomitant with Wegener's granulomatosis is extremely rare: so far, only four cases have been published. The authors report a fifth case where the disease appeared during the post-partum period, a situation which has already been noted in two of the published cases. Interruption of a subsequent pregnancy was followed by a flare-up of the disease resulting in the patient's death. This suggests that post-partum and post-abortum are probably instrumental in the onset and deterioration of Wegener's granulomatosis. The two patients previously reported who received immunosuppressants combined with corticosteroids had no flare-up after delivery. It seems permissible to prescribe such a therapeutic combination before and after delivery or abortion, especially since the fear of foetal toxicity from these drugs seems to be exaggerated.

A case of primary localized amyloidosis of the bladder in a 60 years old male is described. The initial symptom was hematuria. Cystoscopy revealed a redish angioma-like lesion on the trigone, and the diagnosis was pointed out with biopsy, complementary data, and a close follow-up during two years. In the literature we found 47 cases of primary localized amyloidosis of the bladder and 10 others cases of amyloidosis of the bladder which were part of generalized amyloid disease. The interest of the Wright's special staining method is underlined specifying, on light microscopic study, the AA or AL type of the amyloid disease.

The angiofollicular lymphoid hyperplasia, first described in 1954 by Castleman in the mediastinum, is a quite rare pseudolymphoma where there are few subcutaneous localizations. Since 1954, more than 300 observations were published including mediastino-pulmonary forms (about 60 p. 100 of the cases) intra-abdominal forms (15 p. 100 of the cases) and superficial forms which represent 25 p. 100 of the cases and associate superficial ganglionic, intra-muscular and subcutaneous localizations. The authors report the observation of a 44-year-old negro who had a subcutaneous tumefaction of the left elbow which appeared recently without a functional sign nor a biological change. The histological findings allowed the diagnosis of Castleman's pseudolymphoma in a hyalino-vascular form or Flendrig's type II. The evolution was marked a few weeks later by a local recurrence of which a second surgery has secured the recovery. The detailed study of the 76 cases of Castleman's superficial pseudo-tumours published in the literature allows us to recall the features of this disease which affects especially the young adult without prevalence of sex at about 25 years old. The circumstances of discovery are univocal, isolated palpable subcutaneous tumefaction in most of the cases. The localizations are distributed by decreasing incidence as following: latero-cervical, axillary, sus-clavicular, inguinal, vulvar, abdominal wall, shoulder, arm, forearm with a few bifocal forms. The histological aspect associated a predominant lymphoid population and vessels with fibro-hyalinous wall which morphological variations have permitted to individualize three forms: a plasmocytic form or Flendrig's type I which should be a stage of beginning often associated with hematological changes, a hyalino-vascular form or Flendrig's type II more frequent and a mixed form or intermediary type. The immunofluorescence, histo-enzymology and immunohistochemistry studies reveal a changeable polyclonal plasmocytosis and a predominance of T-suppressors in the lymphocytic population. The histological differential diagnosis of the superficial forms of the Castleman's pseudolymphoma is rarely set with certain lymphoma in case of ganglionic localization. On the other hand isolated subcutaneous localizations must be distinguished of the Kimura's disease and of the angiolymphoid hyperplasia with eosinophils where the vessels have a different morphology. The evolution is favorable in most of the cases and surgical exeresis insures the recovery.(ABSTRACT TRUNCATED AT 400 WORDS)

A 51-year-old man presented with isolated diffuse gingival hypertrophy, the diagnosis of a plasmocytoma being suspected after pathological and immunohistological examinations of a gingival biopsy (light chain lambda plasmocytes). The isolated nature of this diffuse gingival plasmocytoma was confirmed by biological tests and radiological investigations. The evolution of solitary plasmocytomas is variable, and while certain of these lesions remain isolated even after many years, other plasmocytomas, apparently local, are associated with myelomas which either become evident or may sometimes develop. The extremely rare nature of diffuse gingival locations of extra-osseous solitary plasmocytomas is confirmed by a review of the published literature. Studying histological sections by immunofluorescence enables the polyclonal or monoclonal nature of the plasmocytes to be established, this distinction being an important element in the prognosis.

The nevus on nevus is a dischromic lesion with a double component made of a pigmented, pale-brown coloured spot, most often congenital punctuated by macular or discretely papular darker elements, with a usually later setting-up and conventionally characterized by an absence of evolutivity. A series of seven cases is reported of whom three present a very peculiar evolution: A 37 years old man is taking a medical advice for a nevus on a congenital nevus on the right buttock on which appeared later on a blue-coloured, lightly sensitive nodule which clinically calls to mind the diagnosis of a blue nevus: the surgical exeresis is refused by the patient. A little girl, born in 1972, has since her birth a nevus on nevus of her right fore-arm; in 1975 and 1976 appeared successively on this lesion three nodules evocative of Spitz melanoma (fig. 4), which are surgically removed; histologic examination confirms the clinical diagnosis (fig. 5). A woman, aged 38, presents a big nevus on congenital zosteriform nevus of the right lower limb; since 1982, one of the dark elements situated on the lower third of the leg is progressively spreading and becoming polychromic (fig. 6); the clinical suspicious of superficial spreading malignant melanoma is confirmed by the histologic examination. In the literature, the terminology aiming to call this kind of nevic pale brown spot recovered by darker macules is not quite clear, as it was already emphasized by Stewart in 1978 (27). Indeed, numerous different denomination are found in it: "nevus spilus", nevus on nevus", "spotty nevus" "speckled lentiginous nevus", "speckled nevus "spilus".(ABSTRACT TRUNCATED AT 250 WORDS)

Authors present the case of a 59 years old man in whom an intra abdominal mass was discovered fortuitly. A surgical intervention permitted to remove a retroperitoneal mass. The macroscopic features were similar to those of multicystic mesothelioma. A review of literature of the 31 cases published permits to present the characteristics of cystic mesothelioma. This entity was confound during a long time with cystic lymphangioma, from which it was distinguished in 1979 with the use of electron microscopy. Interest of studies in immunochemistry, already unpublished, is demonstrated here for Factor VIII and cytokeratin.