[Intramuscular capillary-venous angioma extending into the infratemporal fossa. Apropos of a case]. / Angiome capillaro-veineux intra-musculaire étendu à la fosse infra-temporale. A propos d'un cas.

A rapidly growing tumor in the temporal area extended into the infratemporal fossa. Imaging findings favored a hypervascularized meningo-sarcoma type extracranial tumor, but the pathology report on the surgical specimen confirmed the diagnosis of capillaro-venous angioma. The therapeutic approach was preoperative hyperselective embolization then access via the temporal and infra-temporal fossa for simple tumor exeresis. Mature angiomas and access via the infra-temporal fossa are reviewed.

Expression of p53 in oesophageal squamous epithelium from surgical specimens resected for squamous cell carcinoma of the oesophagus, with special reference to uninvolved mucosa.

Accumulation of p53 protein has been considered an intermediate biomarker in multistage oesophageal carcinogenesis. The aim of the present study was to investigate p53 expression by immunohistochemistry in 13 thoroughly sampled oesophagectomy specimens from a geographical area with a high oesophageal cancer incidence (Basse Normandie, France). Expression of p53 was looked for in tissue samples of cancer, intraepithelial neoplasia, and uninvolved mucosa. The streptavidin biotin peroxidase complex method was used for p53 immunostaining. p53 expression was found in invasive squamous cell carcinoma in 8 out of 11 cases and in intraepithelial neoplasia in 10 out of 11 cases. In all 13 cases, in uninvolved oesophageal mucosa, expression of p53 was focally present in areas of chronic oesophagitis. Chronic oesophagitis has been regarded by epidemiologists as a precursor lesion for squamous cell carcinoma of the oesophagus. Since oesophageal carcinogenesis is a multistage process, the study of precursor lesions could provide information on the timing of p53 gene abnormalities during oesophageal carcinogenesis. These preliminary data require to be confirmed by molecular analysis of the p53 gene.

DNA cytometric abnormalities in human esophageal squamous intraepithelial and invasive carcinomas.

DNA ploidy abnormalities of 21 archival human esophageal intraepithelial neoplasia samples were assessed, using image cytometry of deparaffinized samples, with reference to invasive squamous cell carcinoma and corresponding uninvolved squamous epithelium. Cytometric parameters investigated were proportion of G0G1 aneuploid cell population, histogram typing, proportion of G0G1 diploid nuclei, coefficient of variation, mean DNA content, crude 5c exceeding proportion, 2c deviation index, malignancy index and grade, and entropy. The distributions of the above parameters were compared using the paired t test and Fisher's exact test. Among 10 parameters used, Auer typing of DNA histograms, crude 5c exceeding rate, 2c deviation index and malignancy grade according to Böcking allowed discrimination between uninvolved epithelium and invasive squamous cell carcinoma as well as intraepithelial neoplasia. In particular, the distribution of 2c deviation index in the uninvolved epithelium did not overlap that of intraepithelial and invasive carcinomas. The above four parameters, however, were unable to discriminate intraepithelial neoplasia from invasive carcinoma.

Pathologic assessment of tumor regression after preoperative chemoradiotherapy of esophageal carcinoma. Clinicopathologic correlations.

BACKGROUND: The benefits of preoperative chemotherapy and radiation for esophageal carcinoma are under investigation. A pilot study was undertaken to determine if pathologic assessment of tumor regression correlated with disease free survival. METHODS: Ninety-three resected specimens from patients treated with cis-dichloro-diamino cisplatin and irradiation before surgery were examined on semiserial sections. Patients selected for surgery were all Status 1 according to the World Health Organization (WHO) classification. Histologic typing was based on the WHO classification. Tumor regression grade (TRG) was quantitated in five grades: TRG 1 (complete regression) showed absence of residual cancer and fibrosis extending through the different layers of the esophageal wall; TRG 2 was characterized by the presence of rare residual cancer cells scattered through the fibrosis; TRG 3 was characterized by an increase in the number of residual cancer cells, but fibrosis still predominated; TRG 4 showed residual cancer outgrowing fibrosis; and TRG 5 was characterized by absence of regressive changes. Survival curves were estimated according to the Kaplan-Meier method. A quantification of the relationship between treatment failure and confounding variables (age, tumor location, tumor size, esophageal wall involvement by residual cancer and/or regressive changes, histology, treatment, adequacy of surgery, pathologic lymph node status, and tumor regression grade) was done using Cox's proportional hazards model. RESULTS: Forty-two percent of specimens were TGR 1-2; 20%, TGR 3; and 33%, TGR 4-5. Univariate analysis found that tumor size, pathologic lymph node status, tumor regression grade, and esophageal wall involvement were highly correlated with disease free survival (P < 0.05). After multivariate analysis, only tumor regression (i.e., TRG 1-3 versus TRG 4-5) remained a significant (P < 0.001) predictor of disease free survival. CONCLUSIONS: This study highlights the importance of tumor regression in the survival of patients with esophageal carcinoma treated with preoperative chemoradiotherapy. These findings suggest that tumor regression grade should be considered when evaluating therapeutic results.

[Metatypical carcinoma. Apropos of 4 cases]. / Carcinomes métatypiques. A propos de quatre observations.

The authors describe the anatomopathological, clinical and evolutive characteristics of the metatypical carcinoma, based on four cases and on the existing literature. Evolution of these tumors, with a maxillo-facial preferential location, is usually longer than for other cutaneous carcinomas. Cases presented in this article illustrate the severity of such lesions, whose treatment requires extensive amputations and rather unusual reconstruction procedures.

[Squamous cell carcinoma and disseminated superficial actinic porokeratosis]. / Carcinome spinocellulaire et porokératose actinique superficielle disséminée.

Disseminated superficial actinic porokeratosis was first described by Chernosky and Anderson in 1969. It is characterized by multiple small keratotic lesions on sun-exposed areas beginning in the third of fourth decade. The development of a squamous cell carcinoma within lesions of porokeratosis or the association of superficial actinic porokeratosis with immunosuppression have been well documented. We report the case of a 68-year-old patient who presented actinic porokeratosis associated with rapidly evolutive squamous cell carcinoma of the leg. During the hospitalization, an IgA myeloma was discovered. The authors discuss the relationship between porokeratosis, immunosuppression, and squamous cell carcinoma. Pathogenesis of the lesions is interesting because it is admitted that a keratinocyte clone which carries the porokeratosis abnormality is going to proliferate because of immunosuppression, trauma and infectious diseases. It seems important to search for immunosuppression in patients presenting porokeratosis because the incidence of malignant transformation may increase.

Luteinizing hormone regulation by sex steroids in men with germinal and Leydig cell tumours.

OBJECTIVE: We examined the gonadotrophin secretion in patients with increased plasma concentrations of testosterone and oestradiol due to hCG-producing tumours. DESIGN: Comparison of plasma gonadotrophin concentrations before and after stimulation by GnRH, in eight men with hCG-producing tumours resulting in increased testosterone and oestradiol plasma levels, and in 29 men with Leydig cell tumours resulting in increased oestradiol and normal to low testosterone plasma levels. PATIENTS: Eight men with hCG-producing tumours (six with testicular tumours, two with extratesticular tumours), 29 men with Leydig cell tumours and 15 normal men. The six men with germinal cell tumours of the testis were studied before and after unilateral orchidectomy. MEASUREMENTS: Plasma concentrations of hCG, testosterone and oestradiol were measured before and after intramuscular injection of hCG. LH and FSH were measured before and after intravenous injection of 100 micrograms GnRH. RESULTS: Plasma LH and FSH concentrations were low in patients with germ cell tumours, who exhibited increased plasma testosterone and oestradiol concentrations, and were normal in patients with Leydig cell tumours, in whom oestradiol only was increased. Plasma LH and FSH were normalized in the five patients with successful (e.g. normal hCG, testosterone and oestradiol) unilateral orchidectomy. Basal plasma testosterone concentrations correlated positively (P < 0.01) with plasma oestradiol concentrations in patients with germ cell tumours and negatively (P < 0.01) in patients with Leydig cell tumours. CONCLUSIONS: In patients with hCG-secreting germ cell tumours complete suppression of plasma LH and FSH with increased plasma concentrations of both testosterone and oestradiol are often discovered. No such gonadotrophin suppression is found in patients with Leydig cell tumours, but the negative correlation observed between plasma testosterone and oestradiol in these patients suggests a weak negative feedback effect of oestradiol on LH secretion, which cannot be demonstrated by basal LH measurements in plasma.

[Pseudotumoral hyperplasia of Brunner's glands]. / Hyperplasie pseudo-tumorale des glandes de Brünner.

Hyperplasia of Brünner's glands is a dysembryoplastic or hyperplasic lesion with an elective location of the proximal duodenum. Symptoms are often non-specific but severe manifestations can occur (haemorrhage, duodenal obstruction). Two cases of hyperplasia of Brünner's glands were reported due to their particularly large size and a misguiding clinical appearance. Difficulties in diagnosis due to this rare benign entity were discussed.

[An unusual cause of fracture of the femoral neck: bone sarcoidosis]. / Cause inhabituelle de fracture du col du fémur: la sarcoïdose osseuse.

A case of pathological fracture of the femoral neck in a 84-year old female is presented. The histological examination of the femoral head showed sarcoidosis. This diagnosis was not known before the trauma. Other localisations of bone sarcoidosis were found in the tarsus and the fingers after a systematic X-ray check-up.

[Polyarthritis revealing hairy cell leukemia]. / Polyarthrite révélatrice d'une leucémie à tricholeucocytes.

A female patient simultaneously developed hematologic evidence of hairy cell leukemia and marked but short-lived inflammatory involvement of a number of joints. Both these groups of symptoms resolved simultaneously and rapidly under alpha-2 interferon therapy. This course suggests that the arthritis was a rheumatologic manifestation of the hematologic disease. The concomitant occurrence in this patient of arthritis, splenomegaly and leukopenia was suggestive of Felty syndrome: these two conditions need to be differentiated.

Polycythemia and steroid overproduction in a gonadotropin-secreting seminoma of the testis.

While investigating the cause of mild polycythemia in a young man, a testicular seminoma was discovered with unusual and tumor-dependent features: an absolute polycythemia with high plasma erythropoietin (EPO) levels, an overproduction of estradiol and testosterone, and a dramatic Leydig cell hyperplasia surrounding the tumor tissue. The authors attempted to gain insight into the relationship between this testicular tumor and the hormonal overproduction, i.e., of EPO, estradiol, and testosterone. Their results favored the conclusion that the high EPO levels and the polycythemia were an indirect effect secondary to the steroid overproduction rather than a direct EPO-producing activity. Moreover, the steroid overproduction by the testis could be caused by a paracrine mechanism through human chorionic gonadotropin activity on the Leydig cells.

[Acute megakaryoblastic leukemia. Relation to trisomy 21]. / Leucémie aiguë mégacaryoblastique. Relation avec la trisomie 21.

Two cases of acute megakaryoblastic leukemia in a 4 month-old and a 13 year-old girl are described. In the first case who presented with a large hepatomegaly and portal fibrosis, the diagnosis was made from the surface phenotyping of megakaryoblasts; a trisomy 13, 14 and 19 and an extra chromosome X were present in the bone marrow. An electron microscopy study of megakaryoblasts was necessary to identify the second case. Both children died shortly after treatment (cytosine-arabinoside at low dosage in the first case and polychemotherapy in the second). The 51 other cases reported in the literature are reviewed.

[Cystic chondromalacia of the auricle. A case report. Review of the literature]. / Chondromalacie kystique du pavillon de l'oreille. A propos d'un cas. Revue de la littérature.

Cystic chondromalacia is a clinical and histopathological entity which can be clearly distinguish from all other cystic lesions of the auricle. We report a case which was clinically asymptomatic and involved the scaphoid fossa of the anterior surface of the pinna. The pathological process consisted of degenerative changes of the auricular cartilage which produced a cavity containing a serous fluid. No etiologic factor was found, in particular no trauma. The posterior wall of the cyst was excised under local anesthesia. This case allowed us to review the clinical, histopathological features and surgical difficulties of this rare lesion.

[Spinal lipoma associated with a neuromuscular hamartoma. Report of one case]. / Lipome spinal associé à un hamartome neuromusculaire. A propos d'un cas.

A 6 year-old boy with urinary incontinence, sensory loss and spastic weakness in lower limbs underwent surgical repair for low-lying spinal cord ending in an intradural lipoma. Within the lipoma, bundles and fascicles of striated muscles fibers were intimately associated with nerve fibers. This extremely rare histological appearance has been reported as benign "triton tumor". Our case allows a discussion of its histogenesis.

Prognostic factors in soft tissue sarcomas. A multivariate analysis of 109 cases.

Prognostic factors were evaluated in 109 soft tissue sarcomas of the extremities, walls of the trunk, head, and neck. All lesions were graded according to the systems proposed by the National Cancer Institute (NCI) and the French Federation of Cancer Centers (FNCLCC), and a correlation was found between tumor grade and prognosis. Univariate analysis selected the following variables as unfavorable prognostic factors: invasive tumor margins, extra-compartmental status, deep tumors, tumor diameters over 5 cm, inadequate excision, presence of necrosis, high mitotic count, histologically undifferentiated tumors, and blood vessel invasion. These variables were found to be interdependent. Multivariate analysis selected quality of surgery as the most important variable for predicting local recurrences. The factors selected with regard to overall and metastasis-free survival were tumor size, tumor margins, necrosis, and adequacy of excision. These results permitted classification of patients into four prognostic groups: two with good and two with bad prognosis. Five-year survival for the four groups was 100%, 83%, 53%, and 0%; 5-year metastatic rates were 0%, 12%, 67%, and 100%. Similar groups were obtained when the variables of tumor margins and size were combined with an adaptation of the NCI grading (low-grade tumors/high-grade tumors without necrosis/high-grade tumors with necrosis). Comparative analysis showed that patients with tumors of the same histologic grade or type were not necessarily classed in the same prognostic groups. A better clinicopathologic correlation was obtained using a combination of prognostic factors than with histologic grading or typing alone.

Massive systemic amyloidosis associated with light-chain deposition disease.

A 72-year-old woman presented with rapidly progressive renal failure and multiple myeloma. The patient died 6 months later of severe hepatic insufficiency. The light-microscopic, immunological and ultrastructural findings showed widespread kappa-light-chain deposits including the kidneys, liver, spleen, heart, lungs, tongue, ovary, pancreas and bone marrow associated with massive AL amyloid deposits in the same organs and in the thyroid gland. The concurrent presence of two different deposits is very unusual and the possible mechanisms for such an association are discussed.