[Calciphylaxis: a rare differential diagnosis of systemic vasculitis]. / La calciphylaxie: un diagnostic différentiel rare de vascularite systémique à ne pas méconnaître.

INTRODUCTION: Calciphylaxis is a rare phenomenon of medium- and small-vessel calcifications leading to cutaneous necrosis mimicking vasculitis. CASE REPORT: A 75 year-old-woman with chronic renal insufficiency was admitted for extensive cutaneous necrosis of the limb. Diagnosis of vasculitis was made, but inspite of corticosteroid therapy, the condition of the patient was worsening. After cutaneous biopsy, the diagnosis of calciphylaxis was established. CONCLUSION: Calciphylaxis must be suspected in cases of cutaneous necrosis occurring in a patient with chronic renal failure. Treatment requires rapid normalization of phosphocalcic balance. It is a condition with high mortality.

[Kikuchi-Fujimoto disease: histiocytic necrotising lymphadenitis]. / Maladie de Kikuchi - Fujimoto: lymphadénite histiocytaire nécrosante.

BACKGROUND: Kikuchi-Fujimoto disease or necrotizing histiocyte lymphadenitis is a disease of unknown cause whose main clinical feature is adenopathy, generally in a cervical location. The disease was described for the first time in Japan in 1972 and the first case in France was reported in 1986. Clinicians should be aware of this uncommon disease in order to differentiate it from infectious adenitis, lymphoma and collagenosis. CASE REPORT: A 21-year-old woman consulted the emergency physician in January 2002 for a right cervical mass which had developed rapidly with no associated symptom. Other smaller masses developed within the following days. Laboratory results demonstrated discrete inflammation. All serologies tested were normal. Diagnostic cervicotomy was performed. The histological diagnosis was necrotizing adenitis. DISCUSSION: Necrotizing histiocyte lymphadenitis is generally observed in young women in their twenties. There has been no predominant ethnic background. Laboratory tests are normal excepting for discrete signs of inflammation. Diagnosis is provided by pathology and immunohistochemistry. The clinical course is generally spontaneously favorable in six months. Relapse is uncommon.

Histomorphological characteristics of gastric mucosa in patients with Zollinger-Ellison syndrome or autoimmune gastric atrophy: role of gastrin and atrophying gastritis.

The role of gastrin in the pathophysiology of two diseases affecting the human stomach, the Zollinger Ellison syndrome (ZES) and the pernicious anemia (PA), is reviewed. Both diseases present chronic hypergastrinemia but from different origins. The ZES is characterized by the occurrence of ectopic endocrine gastrin-secreting tumors and PA by a fundic atrophic gastritis leading to complete atrophy of fundus and resulting in achlorhydria. In PA, the lack of acid induces continuous gastrin cell activation and is responsible for the subsequent gastrin hypersynthesis and secretion. In ZES, hypergastrinemia causes hypertrophy of the oxyntic mucosa, which, in addition, displays hyperplasia of parietal and mucus cells. In both diseases, hypergastrinemia also induces the hyperproliferation of enterochromaffin-like endocrine cells in the fundic mucosa, which can offer all aspects from hyperplasia, then dysplasia, until true carcinoid tumor. The influence of antisecretory treatments and MEN 1 in the ZES as well as that of several other factors and antrectomy in PA on the behavior of the different gastric cells is evoked. Finally, the role that gastrin and its receptor play in the maintenance of the normal development of gastric mucosa and gastric acid secretion is emphasized by results observed in gene knockout models.

[Epithelioma cuniculatum. Apropos of a case in the anterior gingiva with involvement of the mandibular symphyseal bone and reconstruction using a fibular osteocutaneous flap and integrated implants]. / Epithelioma cuniculatum. A propos d'un cas gingival antérieur avec extension osseuse mandibulaire symphysaire et reconstruction par lambeau ostéo-cutané fibulaire et pose d'implants.

We present a case report of an anterior gingival epithelioma cuniculatum in place of teeth 41 and 42, with osseous mandibular extension for which a one time reconstruction with an osteocutaneous free fibula flap was performed with three dental osseointegrated implants. We reviewed the literature on this type of carcinoma and discuss the diagnosis difficulty. Radical surgical treatment is required.

[Venous hemangioma of the temporal muscle. Review of the literature: apropos of a case]. / Hémangiome veineux du muscle temporal. Revue de la littérature: à propos d'un cas.

A 41-year-old woman progressively developed a painful tumefaction in the left temporal fossa. The scan showed a isodense intramuscular structure weakly enhanced by contrast medium. Surgical exeresis was performed via the coronal access. Histology of the surgical specimen reported the diagnosis of venous muscular haemangioma. The diagnosis and management of these rare tumours is discussed.

Helicobacter pylori infection: physiopathologic implication of N alpha-methyl histamine.

BACKGROUND/AIMS: In the gastric mucosa of Helicobacter pylori-infected subjects, we previously detected N alpha-methyl histamine (N alpha-MeHA), a minor catabolite of histamine and a potent agonist of histamine H3 receptors. The origin of N alpha-MeHA and its effects on gastric histamine and somatostatin in infected subjects were investigated. METHODS: Ten noninfected patients and 13 patients with intense colonization were compared. N alpha-MeHA content and its synthetic enzyme activity, N alpha-histamine methyltransferase, binding of [3H]N alpha-MeHA, histamine and somatostatin contents, and histidine decarboxylase activity were assayed in antral and fundic biopsy specimens and in cultured H. pylori strains. RESULTS: Gastric histamine and somatostatin contents as well as histidine decarboxylase activity were decreased in infected patients and were restored to normal after antimicrobial treatment. Both N alpha-MeHA and N alpha-histamine methyltransferase activity were present in the mucosa of infected patients and in cultured strains and were very low in noninfected patients or after eradication of H. pylori. [3H]N alpha-MeHA bound to gastric mucosa but not to cultured strains. The [3H]N alpha-MeHA specific binding sites were characterized as H3 receptors. The amount of bound [3H]N alpha-MeHA seemed correlated positively with somatostatin content and histidine decarboxylase activity and negatively with N alpha-MeHA content and N alpha-histamine methyltransferase activity. CONCLUSIONS: H. pylori is the main source of gastric N alpha-MeHA that may lower histidine decarboxylase activity and somatostatin content through H3 receptors.

Seven years of recurrent severe strongyloidiasis in an HTLV-I-infected man who developed adult T-cell leukaemia.

OBJECTIVE: Human T-cell leukaemia/lymphoma virus type I (HTLV-I) is endemic in Japan, the Caribbean basin and Africa, where it has been aetiologically linked to certain chronic myelopathies and adult T-cell leukamia (ATL). We sought to investigate whether strongyloidiasis, a parasitic disease common in these areas, might be a cofactor in the pathogenesis of ATL, as some reports have suggested. PATIENTS, PARTICIPANTS: One 35-year-old HTLV-I-seropositive French West Indian man with a 7-year history of recurrent strongyloidiasis associated with episodic hyperinfestation presenting at the Centre Hospitalier Intercommunal, Villeneuve St Georges, France. INTERVENTIONS: Treatment with various chemotherapeutic agents and symptomatic therapy for hypercalcaemia and antiviral therapy (zidovudine and interferon). RESULTS: The patient developed ATL and died shortly after, despite chemotherapy. Immunological and virological studies performed during the last 15 months of his life showed an increase of the percentage of peripheral ATL cells, and progression from a polyclonal to a monoclonal integration of HTLV-I proviral DNA in the peripheral blood mononuclear and lymph-node cells. CONCLUSIONS: Recurrent strongyloidiasis appears to have been a possible cofactor associated with progression from healthy carrier state to ATL in our patient.

First heterotransplantation of a human carcinoid tumor into nude mice.

The first successful heterotransplantation of a human carcinoid tumor into nude mice is reported. CSH, a voluminous hepatic metastasis of a primary bronchial carcinoid tumor (CSB) was resected and transplanted into three irradiated nude (Swiss-nu/nu) mice both by subcutaneous (SC) and intramuscular (IM) routes; the success rate was five of six. Heterotransplanted tumors took 4 to 5 months to appear in the mice and 1 month to attain a width of 0.5 cm. Both human and mouse tumors (named CSH-SC and CSH-IM) were studied by light and electron microscopy. They were Grimelius-positive, neuron-specific enolase-positive, and bombesin-negative by immunocytochemistry. Furthermore, CSH-SC cells presented characteristic (pear-shaped, rod-shaped, or tadpole-shaped) neurosecretory granules. Although CSB and CSH were slightly serotonin positive by immunocytochemistry, only a few serotonin-positive cells were found in CSH-SC and none in CSH-IM, suggesting partial loss of differentiation or an increase in serotonin catabolism during transplantation.

Evolution of fundic argyrophil cell hyperplasia in nonantral atrophic gastritis.

Fundic argyrophil cells were studied for a mean period of 68.7 months (range, 11-170) in 18 patients with fundic atrophic gastritis and achlorhydria. Initially, 12 patients had hyperplasia of the argyrophil cells, the severity of which was assessed using a semiquantitative classification based on the number of argyrophil clusters per square millimeter. At the end of the study, the degree of hyperplasia was unchanged in 9 patients, had decreased in 2, and had increased in 1; no significant increase in the number of argyrophil clusters, precarcinoid changes, or carcinoid tumors were observed and the high level of gastrinemia [mean, 4.8 (range, 1.9-8.1) times the upper limit for normal) did not change significantly. Of the 6 patients with no hyperplasia at the outset of the study, 4 continued without hyperplasia and 2 presented a low-grade hyperplasia at the 20th and 130th month. Gastrinemia increased significantly in the last patient and stayed normal in the other 5. This study argues in favor of the stable appearance of fundic argyrophil cells in patients with atrophic gastritis and stable gastrinemia.

Sarcoidosis of the nose: report of a case with nasal perforation.

We report a case of multisystem sarcoidosis with involvement of the nose and septal perforation in a young West Indian woman. We discuss the etiology of nasal perforation and prognosis of such a lesion.

Circulating gastrin, endocrine cells, histamine content, and histidine decarboxylase activity in atrophic gastritis.

Thirty-five patients with fundic atrophic gastritis and achlorhydria were classified in two groups according to the presence or absence of fundic argyrophil, mostly enterochromaffinlike cell hyperplasia. Among the biologic and histologic parameters studied, the hyperplasic group differed only by a circulating hypergastrinemia and an antral G-cell hyperplasia. The histamine content, the histidine decarboxylase activity, and the mast cell number of fundic biopsies were determined in 10 controls, 16 of the preceding patients (11 with and 5 without fundic argyrophil-cell hyperplasia), and 5 patients with fundic atrophic gastritis and neither achlorhydria nor hyperplasia. Histamine content and histidine decarboxylase activity were increased only in the hyperplasic group despite an unchanged mast cell number. For all fundic biopsies the argyrophil-cell density was positively related to the histamine content. Finally, the argyrophil-cell hyperplasia occurring in fundic atrophic gastritis with achlorhydria is associated not with the gastritis intensity, as assessed by histologic and secretory criteria, but with a circulating hypergastrinemia and an increase of both fundic histamine content and histidine decarboxylase activity.

[Papillary cystadenocarcinoma of the submaxillary gland. A rare diagnosis]. / Le cystadénocarcinome papillaire de la glande sous-maxillaire. Un diagnostic rare.

The authors describe a case of a "separate" type of salivary adenocarcinoma-like tumor. The growth is described as a papillary cystadenocarcinoma of the submaxillary gland, which should be distinguished from degenerate adenolymphoma. This malignant tumor, which remains "enclosed" until late, grows slowly and should be checked regularly over a protracted period of time following surgical excision performed in accordance with cancer therapy specifications.

Treatment of inflammatory breast cancer with combination chemotherapy and mastectomy versus breast conservation.

Twenty-six consecutive patients with nonmetastatic inflammatory breast cancer (IBC), were treated in a single institution using the same protocol, and all were followed for at least 48 months. The first phase of treatment consisted of two monthly cycles of combination chemotherapy with Adriamycin (Adria Laboratories, Columbus, OH), vincristine, cyclophosphamide and 5-fluorouracil. Local treatment was then undertaken using in all cases a cobalt 60 beam to deliver 45 Gy to the entire mammary gland and lymph-draining areas. Local treatment was completed either by mastectomy, or by conservation of the breast and interstitial irradiation of the primary tumor site. Chemotherapy was resumed after completion of local treatment for a total of 6 cycles. Metastatic disease occurred in 19 of 26 patients from 8 to 55 months; five patients are alive and free of disease from 48 to 81 months. Failure to control local disease or local recurrences was noted in two of ten patients undergoing mastectomy, and in seven of 13 patients with conservation of the breast. While this difference is not statistically significant we concluded that methods of breast conservation which limit the high dose volume to the tumor site do not assure local control in IBC. The median disease-free survival and overall survival of 12 and 31 months, respectively, are not satisfactory. Better systemic treatment is needed.

[Extra-osseous mandibular ameloblastoma]. / Améloblastome mandibulaire extra-osseux.

Gingival ameloblastoma is a rare odontogenic tumor which shares the same histology as endo-osseous ameloblastoma but from which it differs in terms of its course by the absence of "local malignancy". There would appear to be two probable origins concerning this lesion: Serre debris and the epithelium of the gingival mucosa. However, the etiopathogenesis remains controversial, raising problems of pathological distinction from gingival basal cell carcinoma.

Asymptomatic encephalic intraparenchymatous neuroepithelial cysts.

The authors report three cases of intraparenchymatous neuroepithelial cysts, which did not communicate with the ventricular system and the subarachnoid space. They were located in (1) the right frontal lobe, (2) the left cerebral peduncle and pons, and (3) the right cerebellar hemisphere. All of them were asymptomatic, despite their volume and location, and presented as incidental autopsy findings. A developmental origin is likely for these non-neoplastic cysts.

[10-year retrospective analysis of the sequelae of loco-regional treatment of breast cancer]. / Analyse rétrospective à 10 ans des séquelles du traitement loco-régional des cancers du sein.

With a minimum follow-up period of 10 years, the incidence of sequelae after loco-regional treatment of 349 breast cancers remains low, being dominated by lymphoedema. Rare after treatment by irradiation alone, lymphoedema appears more frequently in patients treated by radio-surgical association, in spite of a moderate dose of radiation. If a rigorous technique is carried out, the possibility of achieving satisfactory local control in the axilla with radiation alone without neurological sequelae, therefore brings into question the need for axillary dissection without clinically significant adenopathy.