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Biomedical subjects

J M Joujoux

Publications and source records attributed to J M Joujoux.

At least 19 recordsLinked to original sources

[Cutaneous angiosarcoma of the breast after treatment with conservative surgery and radiotherapy of breast carcinoma].

Secondary cutaneous angiosarcoma of the breast is uncommon. This tumor develops after conservative treatment of breast cancer. We present a case that developed seven years after conservative treatment with surgery and radiotherapy for breast cancer. Several factors may be implicated in the pathogenesis of this tumor (carcinogenic effect of radiotherapy, HHV8 infection), but the essential factor appears to be chronic edema. Prognosis is poor but initial wide surgical resection can achieve prolonged remission. Early diagnosis and treatment are the main prognostic factors.

Aged↗

An association between autosomal dominant cerebral cavernomas and a distinctive hyperkeratotic cutaneous vascular malformation in 4 families.

Cerebral cavernomas (CCMs) are vascular malformations that may be inherited as an autosomal dominant condition for which a gene, CCM1, was mapped to chromosome 7. Poorly defined cutaneous malformations were sometimes described in association with CCMs. During a national survey, 57 French CCM families were studied. Co-occurrence of CCMs and a distinctive cutaneous vascular malformation was observed in 4 families. Ten individuals belonging to these families showed similar hyperkeratotic cutaneous capillary venous malformations (HCCVMs). In 3 families, the histology showed orthokeratosis and hyperkeratosis as well as dilated capillaries in the dermis extending to the hypodermis and confirmed the diagnosis of HCCVM. Genetic analysis strongly supports linkage of these families to the CCM1 locus on chromosome 7. The HCCVM seems to be a peculiar cutaneous vascular malformation associated with CCMs. These data strongly suggest that HCCVMs and CCMs in these families are due to the same genetic abnormality.

Adult↗

[Multiple congenital smooth-muscle hamartomas].

BACKGROUND: Smooth muscle hamartoma is an uncommon lesion. Diagnosis is usually made at birth in infants presenting a plaque with minimal or no infiltration and covered with long dark hairs. Congenital forms with multiple plaques are rarely reported. CASE REPORT: A 5-day-old infant (normal pregnancy and delivery) had plaques localized on the buttocks, the left thigh, leg and shoulder and the right ankle. The plaques were minimally infiltrative and covered with long black hairs. Histology examination showed hyperplastic smooth muscle bundles with varying orientation. The diagnosis was smooth muscle hamartoma. The rest of the clinical examination was normal. CONCLUSION: This case of congenital smooth muscle hamartoma showed a particular form with partially regressive multiple plaques.

Biopsy↗

Primary sinusoidal lymphoma of the liver revealed by autoimmune hemolytic anemia.

Primary liver lymphomas usually present with the clinical picture of a liver tumor, and are characterized by a predominantly portal invasion by lymphoid cells of the B-cell phenotype. We report a case of primary sinusoidal lymphoma of the liver, in a 36 year-old male patient, revealed by homogeneous hepatosplenomegaly and infiltration of liver sinusoids by morphologically normal lymphocytes, without destruction of the parenchyma. Immunohistochemistry in paraffin-embedded tissue sections was positive for the pan T-cell marker MTI, weakly positive for UCHLI, and negative for CD3, and B-cell markers were negative; these findings were consistent with the diagnosis of T-cell lymphoma. The clinical, histological and immunological presentation of this lymphoma was similar to that of hepatosplenic gamma delta T-cell lymphoma. Autoimmune hemolytic anaemia preceded the lymphoma. Despite chemotherapy, the patient died 24 months after the initial presentation in the leukemic phase. A better understanding of this exceptional but characteristic entity is required for an accurate and early diagnosis.

Adult↗

[Pneumatosis cystoides of the colon. Apropos of 7 cases].

Seven cases of pneumatosis cystoides intestinalis affecting the colon (PKC) revealed 5 times by a nonspecific symtomatology and 2 times by an occlusion were reported. In three of the patients the disease was found as a primary idiopathic form free of clinical antecedents. In 4 of the patients the pneumatosis was found to be secondary to a pulmonary disease, a gastric ulcer, a connectivite of a corticotherapy. The PKC was generally diagnosed either by barium enema or by computed tomography and less frequently by colonoscopy with deep biopsy allowing differential diagnosis with colonic polyposis. The mechanism and etiology of the PKC were not fully understood. The illness is a benign condition that often responds to a conservative management--i.e. abstention, oxygenotherapy, diet or antibiotherapy- or exceptionally to surgical colonic resection in case of acute complication.

Aged↗

[Acne induced by amineptin: adnexal toxiderma].

INTRODUCTION: Amineptine-induced acne is a classical side effect often encountered after prolonged and excessive use of the antidepressor. CASE REPORT: We report the case of a young female drug addict (benzodiazepines and alcohol) who denied overuse of amineptine. The initial inflammatory aspect of the lesion was unusual as was the histology report. The sebaceous glands were the site of cystic dilatation as would be expected, but in addition the sudoriparous glands showed keratinizing syringometaplasia with areas of neutrophilic eccrine hidradenitis. DISCUSSION: This is the second report of such a case similar to other due to chemotherapy, benaxoprofene or toxic agents for example, and corresponds to direct toxicity to the sudoriparous glands which accumulate amineptine. We therefore propose the term of adnexal toxic drug eruption.

Acne Vulgaris↗

Tubal sterilization by selective catheterization in an animal model.

RATIONAL AND OBJECTIVES: The feasibility of tubal occlusion by selective salpingography was tested in an animal model; three novel occluding materials also were tested for this application. METHODS: Unilateral selective salpingography was performed in three groups of six rabbits; fallopian tubes were embolized with ethanol (group 1), a hydrogel (group 2), or an occluding emulsion (Ethibloc, Laboratoire Princeps, Neuilly sur Seine, France) (group 3). Animals were killed 2 days or 30 days after the procedure, according to randomization; tubal patency and histologic modifications were evaluated. RESULTS: Selective tubal catheterization was obtained in 100% of the cases. In group 1, no satisfactory occlusion was obtained; in group 2, 65% of the tubes were occluded with little histologic damage; in group 3, 80% of the tubes were occluded, but significant inflammation and tissue necrosis were noted. CONCLUSION: Selective salpingography proved a suitable method for gaining access to the fallopian tube and allowed selective injection of occluding materials. More research is needed to determine a suitable occluding material, focusing on biocompatibility and on long-term efficacy.

Animals↗

[Vulvar involvement in Crohn disease].

INTRODUCTION: There are many skin manifestations of Crohn's disease including specific lesions occurring late after digestive signs. These manifestations are also termed metastatic and are rarely located on the vulva. CASE REPORT: We observed a metastatic localization in the vulva of Crohn's disease in a 34-year-old woman with predominantly rectosigmoid disease. The right labia major was greatly enlarged with oedema involving the inguinal cural area. Pustulization and erosive lesions were seen. The course of these lesions was independent of the colonic disease and responded temporarily only to a triple drug combination of metronidazol, prednisolone and minocyclin. CONCLUSION: This case emphasizes the frequency of colonic or rectal manifestations associated with vulval involvement in Crohn's disease. The metastatic lesions follow a course independent of the digestive disease raising therapeutic problems in this localization.

Adult↗

[Cystic dystrophy in an aberrant pancreas. Contribution of computerized tomography and MRI].

Case report of a cystic dystrophia appearing in an ectopic pancreas. The clinical diagnosis was preoperatively suspected by the findings of CT scan revealing a tumor located in the wall of the duodenum and by the data of MRI indicating its cystic and fibrotic structure. A focus of reacting pancreatitis was found in the vicinity of the cystic dystrophia while the remaining pancreas was normal. The surgical treatment was a duodenopancreatectomy, justified by the potential risks of complications.

Cysts↗

[Segmental neurofibromatosis].

Segmental neurofibromatosis is a rare form of the disease characterized by the unilateral location of cutaneous neurofibromas and café au lait spots along one or several dermatomes. It corresponds to the fifth type in Riccardi's classification. We report the 83rd case of segmental neurofibromatosis discovered by chance in an 83-year old woman presenting with isolated cutaneous neurofibromas situated on the right T10-L1 dermatomes. Type five neurofibromatosis is divided into four subgroups according to the uni- or bilateral site of the lesions, the presence or absence of a family history and the association, or lack of, with deep regional or systemic lesions. It is usually not hereditary, but due to a post-zygotic somatic mutation in the primary neural crest. The disease has a favourable prognosis: no case of evolution towards generalized neurofibromatosis has ever been reported.

Aged↗

[Association of a secreting oncocytic tumor of the kidney and a parathyroid adenoma].

An unusual association of a secreting renal oncocytoma and a parathyroid adenoma is described. The high level of hypercalcemia and of blood parathormone (PTH 44-68), partially reduced by nephrectomy and totally normalized by parathyroidectomy, as well as the renal tumor PTH evaluation and the ultrastructural features showing secretory granules in oncocytic cells of kidney, advocate for a double site of PTH secretion. Removal of both tumors permits a complete recovering.

Adenoma↗

[Giant cystadenocarcinoma of pancreatic origin and supra-mesocolic topography].

The authors report a case of pancreatic cystadenocarcinoma developed within a few days as a voluminous cyst of the left hypochondriac region, pushing away the organs of the supramesocolic zone. The tumor removal is easily performed because it was surrounded by a fibrotic capsule and their was no precise attach to the neighbouring organs. Diagnosis is carried out by microscopic examination revealing a tubulo-papillary cystadenocarcinoma of the pancreas. Quasi synchronous apparition of disseminated hepatic metastases advocates for heavy malignancy and justifies intrahepatic chemotherapy.

Antigens, Tumor-Associated, Carbohydrate↗

[Mucinous cystadenocarcinoma of the appendix. Apropos of a case].

A case of mucinous cystadenocarcinoma discovered into a voluminous benign mucocele of the appendix, is presented. The clinical feature is a tumor of the right iliac fossa. The identification of a limited area of epithelial degenerescence in the cyst wall--by peroperative microscopic examination--permits immediate enlargement of the cystectomy by a right hemicolectomy. The prognosis of the tumor is generally excellent as soon as the diagnosis is early and the removal large enough.

Appendiceal Neoplasms↗

Small carcinomas of the pancreas with associated chronic pancreatitis: two case reports.

Two cases of pancreatitis associated with papillary atypical hyperplastic lesions and micro-invasive cancers are reported. They are interesting from an etiopathogenic and diagnostic point of view by again illustrating the problem of transmission between hyperplastic lesions and cancer, together with the difficulties encountered in asserting the certainty of diagnosis. In each observation the association of pancreatic lesions, of all stages of hyperplasia, and of cancer can be observed. This suggests that transmission occurs between those different lesions. In such cases diagnosis of benignity or malignancy is always difficult to assert. No clinical, biological, morphological or even cytological criterion allows one to make a ruling. For this reason excision is recommended by the authors whenever the etiology of pancreatitis remains obscure and especially in the presence of important dystrophic lesions.

Adenocarcinoma↗