The clinicopathologic characteristics of kidney diseases related to monotypic IgA deposits.

Monoclonal gammopathy of renal significance (MGRS) regroups renal disorders caused by a monoclonal immunoglobulin without overt hematological malignancy. MGRS includes tubular disorders, glomerular disorders with organized deposits, and glomerular disorders with non-organized deposits, such as proliferative glomerulonephritis with monoclonal IgG deposits. Since glomerular involvement related to monotypic IgA deposits is poorly described we performed retrospective analysis and defined clinico-biological characteristics, renal pathology, and outcome in 19 referred patients. This analysis allowed distinction between 2 types of glomerulopathies, α-heavy chain deposition disease (5 patients) and glomerulonephritis with monotypic IgA deposits (14 patients) suggestive of IgA-proliferative glomerulonephritis with monoclonal immunoglobulin deposits in 12 cases. Clinicopathologic characteristics of α-heavy chain deposition disease resemble those of the γ-heavy chain disease, except for a higher frequency of extra-capillary proliferation and extra-renal involvement. IgA-proliferative glomerulonephritis with monoclonal immunoglobulin deposits should be differentiated from diseases with polytypic IgA deposits, given distinct clinical, histological, and pathophysiological features. Similarly to IgG-proliferative glomerulonephritis with monoclonal immunoglobulin deposits, overt hematological malignancy was infrequent, but sensitive serum and bone marrow studies revealed a subtle plasma cell proliferation in most patients with IgA-proliferative glomerulonephritis with monoclonal immunoglobulin deposits. Anti-myeloma agents appeared to favorably influence renal prognosis. Thus, potential progression towards symptomatic IgA multiple myeloma suggests that careful hematological follow-up is mandatory. This series expands the spectrum of renal disease in MGRS.

[Micro-invasive vulvar Paget's disease and lymph node metastasis: a same disease?]. / Maladie de Paget vulvaire micro-invasive et métastase ganglionnaire : une même maladie ?

Vulvar Paget's disease is sub-classified into three types based upon its origin. It might be a primary vulvar disease (type 1) or associated with a non-cutaneous adenocarcinoma-rectal, colonic, cervical (type 2) or linked with an urothelial neoplasia (type 3). Type 1lesions must be considered as potentially invasive. Their immunophenotype is CK7+/CK20-. Classically, in case of depth of invasion below 1mm, nodal metastases are exceptional. We report a case of type 1 Paget's disease in a postmenopausal woman with superficial invasion and multiple inguinal nodal metastases.

[Vaginal adenoid cystic carcinoma: a case report]. / Carcinome adénoïde kystique vaginal: à propos d'un cas.

Adenoid cystic carcinoma generally arises from the salivary glands and is rarely found in the female genital tract. Infection with HPV is implicated in this cervical lesion. Differential diagnosis includes adenoid basal carcinoma, polymorphous low-grade adenocarcinoma and basaloid squamous cell carcinoma. Only one case of vaginal localisation was previously described. We report a case of adenoid cystic carcinoma in a 48-year-old woman with previous cervical HPV infection. Histological examination revealed nests of cells with peripheral palisading organisation and glandular lumina containing material produced by the tumor cells.

[Mural nodules of anaplastic carcinoma in bilateral ovarian borderline mucinous cystadenoma: a case report]. / Nodules muraux de type carcinome anaplasique développés dans un cystadénome mucineux borderline ovarien bilatéral. A propos d'un cas.

The occurrence of mural nodules in serous or mucinous ovarian tumors is not frequent but is currently well established. Mural nodules can developed in benign, borderline or malignant tumors. They can be reactive, benign or malignant. In these situations, the nodule may be carcinomatous, sarcomatous or mixed. Thus, the prognosis of the ovarian tumor can be dramatically modified by the presence of this nodule. About twenty cases of mural nodules of anaplastic carcinoma have been described. We report an additional case of two mural nodules of anaplastic carcinoma associated with bilateral ovarian borderline mucinous tumor. This tumor was synchronous with a renal cell carcinoma. We give details about the classification, the differential diagnoses and prognosis of these nodules.