[Role of medical treatment for symptomatic leiomyoma management in premenopausal women]. / Place des traitements médicaux : indication, durée, efficacité, chez la femme porteuse de fibromes utérins symptomatiques en période d'activité génitale.

The most frequent symptom with leiomyoma is menometrorrhagia. However, it can be responsible of pelvic pain, dysmenorrhea or urinary and digestive compression when it is particularly voluminous. These recommandations were made in order to review medical management of fibroids. If no therapy is able to have them disappear, various drugs may reduce their related symptoms. Tranexamic acid, non-steroidal anti-inflammatory drugs and high dose of oestrogen may be useful in the management of acute hemorrhagic disorders. Progestin, such as lynestrenol induces small reduction in leiomyoma volume and moderate increase in hemoglobin level before surgery. Pregnane and nor-pregnane may improve menstrual bleeding in short or mild delays. The use of Gonadotropin Releasing Hormone (GnRH) agonists can reduce menstrual bleeding with hemoglobin recovery. Add-back therapy using tibolone seems interesting since secondary effects encountered with GnRH agonists may be reduced. Levonorgestrel-releasing intrauterine system is proven to reduce increased menstrual bleeding and restore hemoglobin level. Aminoglutethimide and fadrozole have been underevaluated to conclude when letrozole seems as efficient as GnRH agonists to reduce leiomyoma volume and provide less hot flushes. Anastrozol is associated with reduction in leiomyomata volume, pain and menstrual bleeding. Mifepristone reduces the size of uterine leiomyomata, improves symptomatology, but could be associated with development of endometrial hyperplasia. SPRM evaluated in females have shown to improve leiomyoma related symptomatology. Danazol could be useful to reduce leiomyoma related symptoms in short terms. Tamoxifen and raloxifen show modest overall benefit. Because of insufficient data concerning fulvestrant, pirfenidone or interferon, their prescription cannot be recommended in patients with leiomyomata.

Pulmonary necrobiotic nodules: a rare extraintestinal manifestation of Crohn's disease.

The present article reports the case of a 22-yr-old female with new onset Crohn's colitis, anterior uveitis and multiple pulmonary nodules which, on histological examination, were necrobiotic nodules. This is a rare but recognised pulmonary extraintestinal manifestation of Crohn's disease and only the fourth reported case. The present case report is followed by a brief review of the relevant literature.

Necrobiotic xanthogranuloma associated with paraproteinemia and non-Hodgkin's lymphoma developing into chronic lymphocytic leukemia: the first case reported in the literature and review of the literature.

A 56-year-old married female presented in May 1998 with a 5-month history of xanthelasma of the eyelids, followed 4 months later by two enlarged lymph nodes of the left side of the neck and three of the left axilla. At the same time, she developed xanthomatous patches on the face, neck, and shoulders (Fig. 1). The cutaneous lesions were xanthomatous nodules and plaques, affecting the periorbital regions. Later, the whole face was affected, followed by ulcerated lesions on the scalp, chest, back, and extremities (Fig. 2). The skin lesions became painful, pruritic, ulcerated tumors (Fig. 3). In July 1998, computed tomography (CT) scans of the chest and abdomen with contrast medium showed pretracheal, bilateral axillary, right retrochural, paracaval, aortocaval, and para-aortic lymph node enlargement. These findings were suggestive of lymphoma. CT scan also showed slight heterogeneous hypodensity in the upper part of the right lobe of the liver, suggesting fatty infiltration. The spleen, pancreas, and suprarenal glands appeared normal. One cervical and two left axillary lymph nodes were excised. They revealed total replacement of the nodular architecture by a diffuse proliferation of mature lymphoid cells having small nuclei and a crumbled chromatin pattern, and very rare mitosis. It was concluded from the lymph node biopsies that these changes were typical of non-Hodgkin's lymphoma, diffuse and small cell type, of low-grade malignancy. A bone marrow aspirate showed a marrow heavily infiltrated by lymphoid cells with some immaturity. The megakaryopoiesis was adequate. Trephine biopsies showed similar changes. Iron stores appeared to be absent. The bone marrow picture was consistent with diffuse, well-differentiated non-Hodgkin's lymphoma, developing into chronic lymphocytic leukemia (CLL). Endoscopy showed antral-type gastric mucosa exhibiting mild chronic gastritis. Skin biopsy from a fresh lesion on the back showed a diffuse inflammatory cell infiltrate with collections of histiocytic cells. It also showed necrobiotic foci, surrounded by mixed inflammatory cells, dark palisaded foamy histiocytes, and a few Touton giant cells. These findings are compatible with necrobiotic xanthogranuloma (NXG) (Figs 4 and 5). Blood film showed normochromic, normocytic erythrocytes with anisopoikilocytotic leukocytes and normal platelets. The sedimentation rate was 90 mm in the first hour. The blood picture also showed monoclonal IgG paraprotein (3170 mg/dL) of the kappa light chain type. The patient was treated by the oncologist for her lymphoma, and was given Cytoxan, prednisolone, endoxan, Leukeran, and melphalan. She showed an excellent response to pulsed treatment with steroids (60 mg prednisolone orally daily for 5 days, repeated every month for 6 months). She also responded to Leukeran at a dose of 5 mg daily for 5 days every month for 6 months, and showed regression in the size of the lymph nodes. The treatment of her skin lesions was unsatisfactory in spite of the fact that she was given cyclosporine and both systemic and topical corticosteroids.

[Angular pregnancy at eleven weeks gestation on a fibromyomatous uterus]. / Grossesse angulaire diagnostiquée à 11 SA sur un utérus fibromyomateux: à propos d'un cas.

Reported cases of angular pregnancy, which by definition corresponds to pregnancy developing in a uterine horn, are rare. Etiologies as well as the diagnostic and therapeutic strategies are discussed. The anatomic modifications resulting from uterine fibromyoma and affecting embryo nidation in the uterine cavity of the uterus are the leading cause of angular pregnancy. The course of pregnancy depends on the evolution of the fibromyoma. Treatment therefore depends on the clinical course. Early diagnosis is essential for conservative treatment. In the case presented here, angular pregnancy was diagnosis following development of aseptic necrobiosis, the most common complication of fibromyosma.

Necrobiotic palisading granulomas associated with breast carcinoma.

AIMS: The presence of granulomas within the stroma of carcinomas and in the lymph nodes draining carcinomas has been well described. To date, however, there have been few studies examining the occurrence and relevance of necrobiotic granulomas occurring in association with breast carcinoma. METHODS/RESULTS: Four cases of breast carcinoma with necrobiotic granulomas were examined using periodic acid Schiff and Ziehl Neelsen stains for fungi and tubercle bacilli and with immunohistochemistry using CAM 5.2, cytokeratin 7, and cytokeratin AE1/3 for tumour cells. In one case the stroma was involved, in the other three cases the lymph nodes contained necrobiotic granulomas. In two of the cases, one with stromal and one with lymph node involvement, the necrobiotic granulomas contained necrotic tumour cells. CONCLUSION: In this study the features of four cases of breast carcinomas with necrobiotic granulomas are examined and their relevance explored. Close scrutiny of such granulomas is necessary to avoid underdiagnosis of metastatic disease.

Granulomatous bronchiolitis with necrobiotic pulmonary nodules in Crohn's disease.

A 37-year-old man with extensive Crohn's disease of the stomach, small and large intestine for almost a decade developed respiratory symptoms and radiological findings suggestive of pneumonia that failed to resolve with antibiotic treatment. Computed tomography scanning of his lungs showed extensive changes with cavitated parenchymal nodules. Histological evaluation of an open lung biopsy showed granulomatous bronchiolitis and pulmonary necrobiosis. Treatment with steroids and immunosuppression resulted in complete resolution of his clinical symptoms of pneumonia and abnormal computed tomography imaging changes. Granulomatous bronchiolitis and necrobiotic nodules may be a manifestation of Crohn's disease in the absence of microbial agents, including mycobacteria or fungal agents. While a multiplicity of complex pulmonary changes may occur in Crohn's disease, their clinical recognition and precise pathological definition may be particularly important if treatment with a biological agent, such as infliximab, is being considered.

Conjunctival necrobiotic granuloma in xeroderma pigmentosum.

PURPOSE: We report clinical and histopathologic findings of a conjunctival lesion associated with xeroderma pigmentosum. METHODS: A Saudi girl with known xeroderma pigmentosum presented with pain and photophobia of the right eye and an elevated temporally located perilimbal mass. RESULTS: The mass was resected successfully and has not recurred during 1-year follow-up. Histopathologic examination of the tissue showed a necrobiotic granuloma with associated histiocytic infiltration. The patient had no systemic disease, lipid was not detected in the histiocytic lesion, no Touton giant cells were present, and there was no evidence of elastolysis. CONCLUSION: Ocular malignancies occur in association with xeroderma pigmentosum, but benign lesions that mimic a malignancy may occur.

[Necrobiotic xanthogranulomatosis: a cutaneous manifestation of a monoclonal IgM gammopathy]. / Xanthogranulomatose nécrobiotique: manifestation cutanée d'une IgM monoclonale.

INTRODUCTION: Necrobiotic xanthogranulomatosis is a rare cutaneous disorder usually associated with monoclonal gammopathy. Most frequently, cutaneous lesions involve the face (periorbital region) and the trunk, and are characterized by indurated xanthomatous plaques and nodules. EXEGESIS: We describe a patient presenting with necrobiotic xanthogranulomatosis that was unusual, as it was associated with IgM kappa monoclonal gammopathy of undetermined clinical significance and because no periorbital involvement was observed. CONCLUSIONS: Necrobiotic xanthogranulomatosis should be distinguished from other cutaneous manifestations associated with plasma cell dyscrasias, such as normolipemic plane xanthoma. It can be associated with IgM gammopathy.

Vaccine-induced necrobiotic granuloma.

We report two cases of necrobiotic palisaded granulomas which developed at the site of intradermal hepatitis B vaccination. To the best of our knowledge, this kind of reaction has not been reported previously.