Calcified glomus tumor of the shoulder. A case report.

The authors report a case of calcified glomus tumor of the shoulder in a 54-year-old woman. The nonspecific clinical findings and the noncharacteristic imaging results made diagnosis of this tumor impossible before surgery. The diagnosis was confirmed by a biopsy. The outcome after surgical resection was excellent.

[Langherans' cell histiocytosis: a case report]. / Histiocytose X: à propos d'un cas.

Langherans' cell histiocytosis is rare in adults. It should be considered in patients with lytic osseous lesions with sclerotic rim especially when involvement of adjacent soft tissues is present.

A nationwide study of granulosa cell tumors in pre- and postpubertal girls: missed diagnosis of endocrine manifestations worsens prognosis.

BACKGROUND: There are very few data on the natural history of ovarian granulosa cell tumors (OGCT) in children. The aim of this study was to determine whether early recognition and diagnosis of the initial endocrine signs could improve the outcome of these tumors. METHODS: In a nationwide study from 1990 to 2004, we analyzed the clinical, biological and pathologic data from 40 pre- and postpubertal girls presenting an OGCT. RESULTS: 1. Among the prepubertal girls (n = 29), 17 OGCTs were diagnosed on the basis of precocious pseudopuberty. None of the 17 girls had a peritoneal spread of the tumor (100% FIGO stage Ia). Diagnosis based on a tumoral or acute abdomen (12 cases) was associated with frequent intraperitoneal ruptures of the tumor (50%) and a risk of relapse (2 cases). Of the eight girls who had had a misdiagnosed precocious pseudopuberty, five had a pre- or perioperative tumoral rupture. 2. Among the postpubertal girls (n = 11), endocrine manifestations such as secondary amenorrhea or virilization had been underevaluated in three of them and the diagnosis was established from a tumoral abdomen. This clinical presentation was associated with frequent ruptures of the mass in the peritoneum (80%) and a higher risk of recurrence (30%). 3. A delayed diagnosis of OGCT despite previous endocrine signs (11 cases; 8 pre- and 3 postpubertal) was associated with a high risk of pre- or peri-operative peritoneal tumor spreading (70% FIGO stage Ic or IIc, p <0.05). The mean delay for diagnosis ranged from 3 to 11 months. CONCLUSION: This study highlights the critical role of early diagnosis of OGCT in pre- and postpubertal girls, particularly at the first seemingly banal signs of endocrine disorder. Peritoneal spread of the tumor may thereby be prevented, which improves the prognosis.

[Carcinoma with false negative mammogram: when and how?]. / Des cancers du sein sans signe en mammographie: quand et pourquoi?

Breast carcinomas with false negative mammogram correspond to carcinomas diagnosed with means other than mammogram when the mammogram, at the time of diagnosis, showed no significant abnormality. The rate of false mammogram is difficult to ascertain because few studies have been published on this subject. The absence of mammographic abnormality is related to histopathological characteristics of the tumor and mammographic features of the patient's breast tissue. The small size of the tumor, the lack of microcalcifications often due to tumor necrosis and the absence of any significant associated desmoplastic reaction are the main histopathological factors encountered, particularly for dense breasts.

Time-course of tomato whole-plant respiration and fruit and stem growth during prolonged darkness in relation to carbohydrate reserves.

To evaluate the relevance of a simple carbon balance model (Seginer et al., 1994, Scientia Horticulturae 60: 55-80) in source-limiting conditions, the dynamics of growth, respiration and carbohydrate reserves of tomato plants were observed in prolonged darkness. Four days prior to the experiments, plants were exposed to high or low light levels and CO(2) concentrations. The concentration of carbohydrates in vegetative organs was 30-50 % lower in plants that were exposed to low carbon assimilation conditions compared with those exposed to high carbon assimilation conditions. During prolonged darkness, plants with low carbohydrate reserves exhibited a lower whole-plant respiration rate, which decreased rapidly to almost zero after 24 h, and carbohydrate pools were almost exhausted in leaves, roots and flowers. In plants with high carbohydrate reserves, the whole-plant respiration rate was maintained for a longer period and carbohydrates remained available for at least 48 h in leaves and flowers. In contrast, fruits maintained fairly stable and identical concentrations of carbohydrates and the reduction in their rate of expansion was moderate irrespective of the pre-treatment carbon assimilation conditions. The time-course of asparagine and glutamine concentrations showed the occurrence of carbon stress in leaves and flowers. Estimation of source and sink activities indicated that even after low carbon assimilation, vegetative organs contained enough carbohydrates to support fruit growth provided their own growth stopped. The time of exhaustion of these carbohydrates corresponded grossly to the maintenance stage simulated by the model proposed by Seginer et al. (1994), thus validating the use of such a model for optimizing plant growth.

Anaplastic lymphoma kinase (ALK) protein expressing lymphoma after liver transplantation: case report and literature review.

Most post transplantation lymphoproliferative disorders (PTLDs) are Epstein-Barr virus (EBV) associated B cell proliferations. We report a case of aggressive anaplastic large cell lymphoma expressing the anaplastic lymphoma kinase (ALK) protein in a 58 year old man who had previously undergone liver transplantation. A definite diagnosis was not possible on histopathological examination. Immunostaining clearly showed a predominant population of small irregular lymphocytes, admixed with large cells strongly positive for CD30, epithelial membrane antigen, and the ALK protein. Neoplastic cells were of the T/cytotoxic phenotype. In situ hybridisation with EBV encoded early RNA probes showed only a few scattered positive non-neoplastic small lymphocytes. Polymerase chain reaction analysis of immunoglobulin and T cell receptor rearrangements was negative. The NPM-ALK fusion transcript associated with the t(2;5) translocation was detected by reverse transcription polymerase chain reaction. A review of the literature revealed 76 cases of T cell PTLD, showing a broad spectrum of morphological features and clinical behaviour. Most of these cases were EBV negative (61 of 76) and occurred after renal transplantation (48 of 76). To our knowledge, this is the first case of ALK positive lymphoma occurring in the setting of organ transplantation. This observation stresses the need for accurate immunostaining for diagnosing this rare, apparently aggressive, lymphoma in immunosuppressed patients.

Form I Rubiscos from non-green algae are expressed abundantly but not assembled in tobacco chloroplasts.

Non-green algae have Rubiscos that are phylogenetically distinct from their counterparts in green algae and higher plants. Some non-green-algal Rubiscos are more specific for CO2, relative to O2, than higher-plant Rubiscos, sometimes coupled with lower Michaelis constants for CO2. If these Rubiscos could be substituted for the higher-plant enzyme, and if they functioned successfully in the higher-plant chloroplast and were regulated appropriately, they would improve the CO2 use and quantum efficiency of higher-plant photosynthesis. To assess the feasibility of expressing non-green algal Rubiscos in higher-plant chloroplasts, we inserted the rbcLS operons from the rhodophyte Galdieria sulphuraria and the diatom Phaeodactylum tricornutum into the inverted repeats of the plastid genome of tobacco, leaving the tobacco rbcL gene unaltered. Homoplasmic transformants were selected. The transgenes directed the synthesis of abundant amounts of transcripts and both subunits of the foreign Rubiscos. In some circumstances, leaves of the transformants with the P. tricornutum Rubisco contained as much foreign Rubisco protein as endogenous tobacco Rubisco (>30% of the soluble leaf protein). However, the subunits of the foreign Rubiscos were not properly folded and/or assembled. All the foreign large subunits and most of the foreign small subunits were recovered in the insoluble fractions of leaf extracts. Edman sequencing yielded the expected N-terminal sequences for the foreign small subunits but the N-termini of the foreign large subunits were blocked. Accumulation of large amounts of denatured foreign Rubisco in the leaves, particularly of the P. tricornutum transformants, caused a reduction in the amount of tobacco Rubisco present, with concomitant reductions in leaf CO2 assimilation and plant growth.

Unlikely role of Epstein-Barr virus in the pathogenesis of primary cutaneous CD30+ anaplastic large cell lymphoma.

BACKGROUND: Primary cutaneous CD30+ anaplastic large cell lymphoma (ALCL) is a rare subset of cutaneous lymphoma, with a much better prognosis than its nodal counterpart. The pathogenesis of both nodal and primary cutaneous CD30+ ALCL is largely unknown but experimental data support the hypothesis that the Epstein-Barr virus could play a role in the nodal subset. OBJECTIVE: To evaluate the involvement of Epstein-Barr Virus (EBV) in primary cutaneous CD30+ ALCL by searching for both nucleic acids and EBV proteins in cutaneous lesions. SETTING: Two University Hospitals in Southern France (secondary referral hospitals). PATIENTS: Eight consecutive patients with typical primary cutaneous CD30+ anaplastic large cell lymphoma were studied. METHODS: Search for the presence of DNA, RNA and EBV proteins in cutaneous lesions by PCR, in situ hybridization and immunohistochemistry. RESULTS: EBV DNA and RNA was identified in only one lesion of primary cutaneous CD30+ ALCL and in none of the normal adjacent skin samples. In situ hybridization and immunohistological studies were consistently negative in all samples. CONCLUSION: These results do not support an early role of EBV in the oncogenetic pathogenesis of primary cutaneous CD30+ ALCL.

Decreased expression of estrogen receptor beta protein in proliferative preinvasive mammary tumors.

To understand the significance of estrogen receptor beta (ERbeta) in mammary carcinogenesis, we evaluated the expression of ERbeta in preinvasive mammary tumors. The percentage of ERbeta-positive epithelial or tumoral cells was assayed by quantitative immunohistochemistry using an image analyzer in 130 lesions of varying histological risk from 118 patients [71 with benign breast disease (BBD) and 59 with carcinoma in situ (CIS)] and compared with 118 adjacent histologically normal glands. Five groups of lesions with an increasing risk of invasive cancer, from BBD without hyperplasia to high-grade CIS, were studied. Results were compared with ERalpha and Ki67 immunostaining. The percentage of ERbeta-positive cells was high (median, 85%) in "normal" mammary glands and in nonproliferative BBD and decreased significantly (P < 0.0001) in proliferative BBD without atypia and in CIS, contrasting with an inverse progression for the ERalpha level. In normal mammary glands, the ERbeta level did not vary according to the nature of the lesion at the periphery and was significantly higher (P < 0.007) than in adjacent preinvasive lesions, except in nonproliferative BBD. The ERbeta level decreased in proliferative BBD, anticipating the ERalpha increase, which was significant in BBD with atypia. In high-grade ductal carcinoma in situ, both ER levels were low. The ratio between ERbeta and ERalpha was high in normal glands, and decreased significantly in proliferative lesions. ERbeta staining was inversely correlated with Ki67 (r = -0.333; P < 0.001), more particularly in high-grade ductal carcinoma in situ (r = -0.57; P < 0.02). The marked and early decreased level of ERbeta protein associated with other criteria of cell proliferation suggests a protective effect of ERbeta against the mitogenic activity of estrogens in mammary premalignant lesions. Knowledge of the ERbeta and ERalpha content in each preinvasive lesion should help to rationalize antiestrogen preventive therapy adapted to each individual patient.

[Actinomycosis after renal transplantation: apropos of 1 case and review of the literature]. / Actinomycose après transplantation rénale: à propos d'une observation et revue de la littérature.

Actinomycosis is a suppurative infection usually due to a facultative anaerobic bacteria, actinomyces israelii. This rare infection has been reported in immunocompetent individuals, with buccal or pharyngeal mucosal erosions. Paradoxically, few cases have been observed after solid organ transplantation: 2 cases after lung, 1 case after heart-lung transplantation and 1 case after renal transplantation. We report on a renal transplant recipient who developed a tongue and oropharynx suppurative abscess, looking like an epithelioma. Histological examination showed granulomatous inflammation with an angiofibroblastic reaction; few colonies of actinomyces were also observed by the pathologist. This lesion disappeared easily and totally after tetracycline treatment.

[Germ cell tumors of testis, current concepts]. / Tumeurs germinales du testicule, conceptions actuelles.

Histological pleomorphism of testicular germ cell tumors complicates diagnosis. These tumors share highly specific differentiation properties ranging from intra-tubular neoplasia to classical infiltrating lesions. This general review presents the histopathological features and diagnostic criteria of typical forms and atypical variants. In current practice, distinction between seminoma and non-seminomatous germ cell tumors is fundamental for treatment. Histological typing of non-seminomatous tumors may be problematical, especially in "mixed" forms. Vitelline tumor variants and atypical embryonic carcinoma are described. Unlike scattered isolated syncytiotrophoblastic cells, true chorio-carcinoma is rare and must be carefully identified. Such cells are frequently associated with seminoma, embryonic carcinoma and vitelline tumors. Intra-tubular germ-cell neoplasia and gonadoblastoma, are pre-invasive lesions associated with cryptorchidism and gonadal dysgenesis. Teratomatous elements usually occur in mixed germ cell tumors. Rare pure teratoma are benign in children. On the contrary, post-pubertal forms have an unpredictable evolution.

[Association of Kaposi sarcoma--multiple myeloma. A new case]. / Association maladie de Kaposi--myélome multiple. Un nouveau cas.

INTRODUCTION: Kaposi's disease is an angiogenic multifocal cancer process that has several forms, namely Mediterranean, African, HIV-associated, and secondary to a preexisting immunodepressive state (hematological disorder, corticosteroid therapy, immunodepressive treatment). Whatever its form, Kaposi's sarcoma is probably associated with a chronic viral human herpes type 8 infection (HHV8). EXEGESIS: This virus has been implicated in the pathogenesis of multiple myeloma (17 cases recorded to date). In the present study, a further case of Kaposi's sarcoma associated with multiple myeloma has been reported. However, Epstein-Barr virus, cytomegalovirus, hepatitis B and C, HIV and HHV8 serologies were negative. Radiotherapy on the lower limbs was initiated. CONCLUSION: It is concluded that HHV8 does not appear to play a pathogenic role in cases of multiple myeloma, given the rarity of the association between Kaposi's sarcoma/multiple myeloma/HHV8.

Dissociated overexpression of cathepsin D and estrogen receptor alpha in preinvasive mammary tumors.

The role of estrogen as a promoter agent of sporadic breast cancer has been considered by assaying, in benign breast disease (BBD) and in situ carcinomas (CIS), 2 markers, the estrogen receptor alpha (ERalpha) and cathepsin D (cath-D) involved in estrogen action on mammary tissue. ERalpha and cath-D were assayed by quantitative immunohistochemistry using an image analyzer in 170 lesions of varying histological risk (94 BBD and 76 CIS), and in "normal" glands close to these lesions. The ERalpha level increased significantly in proliferative BBD with atypia (P < .001), in non-high-grade CIS (P < .001), and in adjacent "normal" glands. ERalpha level was decreased in high-grade ductal CIS (DCIS) and also in adjacent "normal" glands. Cath-D level increased in ductal proliferative BBD (P < or = .01) and in high-grade DCIS (P < or = .003), but not in the other lesions. After menopause, ERalpha level was increased (P = .012) but not cath-D level. According to Mac Neman test, the high-grade DCIS were predominantly ERalpha negative and cath-D positive (P = .0017), and the other CIS were predominantly ERalpha positive and cath-D negative (P = .0002). The 2 markers are overexpressed early in premalignant lesions, but independently. This dissociation suggests a branched model of mammary carcinogenesis involving 1 estrogen-independent pathway with high cath-D and low ERalpha levels (including high-grade DCIS) and 1 estrogen-dependent pathway, with high ERalpha level (including proliferative BBD with atypia and low-grade DCIS). We propose that ERalpha-negative breast cancers may develop directly from high-grade DCIS and that ERalpha assay in preinvasive lesions should be considered in prevention trials with antiestrogens.

[Sinus histiocytosis (Destombes-Rosai-Dorfman disease) revealed by extranodal spinal involvement]. / Histiocytose sinusale (maladie de Destombes-Rosai-Dorfman) révélée par une atteinte extraganglionnaire rachidienne.

BACKGROUND: Sinus histiocytosis with massive cervical lymphadenopathy (Rosai-Dorfman disease) is a non-neoplastic lymphoproliferative disorder. Extranodal involvement, especially of the nervous system, is unusual. We report a case revealed by neurological symptoms. CASE REPORT: A 10-year-old girl presented with paraparesis due to a dural extramedullary mass on magnetic resonance imaging. Massive cervical lymphadenopathy appeared secondarily. Radiological investigations showed mediastinal, paranasal sinus and lower eyelid involvement. The diagnosis of Rosai-Dorfman disease was established histologically and by immunohistochemical studies of nodal lesions by the demonstration of characteristic sinus histiocytosis with sheets of S-100 protein and CD-68 positive large histiocytes displaying lymphocyte phagocytosis. A dramatic response occurred with complete resolution of all clinical findings after treatment with corticosteroids and etoposide, although neurological lesions were unchanged on magnetic resonance imaging. CONCLUSION: Despite its rarity, this case underlines the unknown pathogenesis of this disease (immune dysfunction?) and the difficulties of treatment (choice of chemotherapeutic agents, duration).

Hepatitis C virus genotypes and quantification of serum hepatitis C RNA in liver transplant recipients. Relationship with histologic outcome of recurrent hepatitis C.

The reasons for wide variations in the severity of recurrent hepatitis C after liver transplantation are unclear. We studied liver transplant recipients to assess the effect of hepatitis C virus (HCV) genotype and HCV RNA quantification on histologic progression of recurrent hepatitis C after transplantation. Twenty-five patients underwent transplantation for HCV cirrhosis and were followed up with virologic and histologic assessments for a mean of 51 months. HCV genotype was determined by line probe assay. HCV RNA was quantitated in serum samples by nested polymerase chain reaction. The HCV genotype 1 was detected in 17 patients and other genotypes in 8. Acute lobular hepatitis developed in 17 patients 162 days posttransplantation on average. Long-term biopsy specimens (mean, 51 months after the date of liver transplantation; range, 24-86 months) showed chronic hepatitis in 19 patients (mild, 5; moderate, 9; and severe, 5, 2 with extensive scarring). The serum alanine aminotransferase level was correlated with hepatocyte necrosis (piecemeal and lobular) but not with portal inflammation or fibrosis. Patients infected with genotype 1 had a higher Knodell score, and the 5 patients with severe hepatitis C all were infected with genotype 1. HCV RNA levels were significantly higher in patients with genotype 1 than in patients with other genotypes, as were the severity of histologic recurrence and levels of viral replication.

[Urogenital amyloidosis: clinico-pathological study of 8 cases]. / Amylose urogénitale: étude anatomo-clinique à propos de 8 cas.

Amyloidosis of the genito-urinary tract is uncommon. We report 8 cases, often misdiagnosed as a neoplastic process (6/8). Amyloidosis was localized in the bladder (3 cases), in the ureter (1 case) and in the prostate and/or seminal vesicles (4 cases). The amyloid protein was characterized in 7 cases by immunohistochemistry. Among the bladder and ureter amyloidosis, 2 cases were classified as AL lambda amyloidosis and one case as AA amyloidosis in a patient with long history of chronic arthritis. In the fourth case, the deposits could not be identified. Nevertheless an AL amyloidosis might be suggested. Two cases of prostate and/or seminal vesicles amyloidosis were stained with an anti-B2M antibody, in hemodialyzed patients. The 2 others, positive with the anti-Transthyretina antibody, were classified as senile amyloidosis. This small series illustrated the heterogeneous pathogenic types of amyloidosis in the urogenital tract and emphasized the interest of immunohistochemistry to identify the chemical composition of these deposits.