Is sclerosing angiomatoid nodular transformation (SANT) of the splenic red pulp identical to inflammatory pseudotumour? Report of 16 cases.

AIMS: To report 16 cases of sclerosing angiomatoid nodular transformation (SANT) of the splenic red pulp. METHODS AND RESULTS: Patients were selected in two phases. An initial group of seven patients was diagnosed with SANT based on the presence of angiomatoid nodules. Sheets of inflammatory fibrosis were found in three patients, resembling inflammatory pseudotumour (IPT); nine further cases of IPT were reviewed. Angiomatoid nodules were detected, leading to the diagnosis of SANT in all cases. The splenic mass (10-150 mm in diameter) was polycyclic, composed of multiple small nodules of loose connective tissue comprising myofibroblasts and a dense network of capillaries as well as some remnants of sinuses. Collagenous fibrosis surrounded them. Bands or large sheets of fibrosis, infiltrated by various inflammatory cells, particularly polytypic plasmacytes, resembling IPT, were present in 10 cases. CONCLUSIONS: SANT of the red pulp is a distinct benign pseudotumorous lesion of the spleen characterized by the presence of angiomatoid nodules. We observed such angiomatoid nodules in all our cases of splenic IPT, which were not follicular dendritic cell or myofibroblastic tumours. We therefore recommend careful examination for angiomatoid nodules in all suspected cases of splenic IPT.

Experimental study on a large animal model of a new thermoablation technique.

BACKGROUND: A novel technique of thermoablation, using a microtube to deliver pulses of hot water vapour, was tested on a large animal model in order to evaluate its efficacy and potential adverse effects. MATERIALS AND METHODS: The medical device consisted of a microtube extension connected to a hydropneumatic pump. Pulses of pure water were injected though the microtube where they were heated and delivered as vapour into the target zone. The method was tested on the liver of 12 healthy pigs, either during open surgery or percutaneously under ultrasounds. RESULTS: The technique was efficient and well-tolerated by the animals. Large volumes of necrotic tissue were created in a significantly short time compared to concurrent thermoablative techniques. CONCLUSION: Anticipating human application, this experimental study demonstrated a safe and efficient innovative thermoablation technique. The first human applications have been successfully performed and will be reported soon.

Endoscopic exploration and lymph node sampling of the axilla. Preliminary findings of a randomized pilot study comparing clinical and anatomo-pathologic results of endoscopic axillary lymph node sampling with traditional surgical treatment.

OBJECTIVE: To describe the technique of endoscopic exploration of the axilla. To compare this technique to open surgical treatment by comparing the following variables: operative time, peri-operative complications, duration of hospital stay, node's histology and morphologic aspects and esthetic results. MATERIALS: Standard instruments for traditional operative laparoscopy plus a lipo-aspirator (0.8 Bar). PATIENTS: Forty patients, 20 (group A) undergoing open surgery and 20 (group B) undergoing axilloscopy. All patients with early invasive breast cancer are eligible for conservative operative treatment. METHOD: Randomized study. The technique is described and preliminary results are presented. RESULTS: The operative time for axilloscopy is approximately double that for open surgery. A comparable number of lymph nodes is collected by axilloscopy and open surgery. The nodes collected by axilloscopy are more likely to be fractured. What is the clinical consequence? Two loco-regional relapses are observed in the endoscopic group. DISCUSSION: Axillary sampling by endoscopic procedure gives the same pathologic information than surgical axillary sampling. Anatomo-pathologic aspects of nodes and possibilities of relapses were two drawbacks of this procedure. CONCLUSION: Operative time is increased for axilloscopy compared with open surgery. The techniques yield comparable anatomo-pathologic results. It is still unknown whether this endoscopic technique is as effective as traditional surgery or if the frequency or severity of lymphedema is decreased by the endoscopic approach.

[Clinical and pathological study of a case of subacute combined degeneration of the cord with folic acid deficiency (author's transl)]. / Etude anatomo-clinique d'un cas de dégénérescence combinée subaiguë médullaire au cours d'une carence en acide folique.

Clinical and pathological findings in a case of subacute combined degeneration of the cord are described. The patient was a severe alcoholic but there was no vitamin B12 deficiency. Lesions were similar to those observed in Lichtheim's disease. Biological tests demonstrated frank folic acid deficiency, with very low serum and red cells levels. Clinical features included only mild macrocytic anemia, and flexion quadriplegia after a period of disorders of equilibrium. Higher functions were unaffected. Apart from alcoholism, no other toxic or digestive causative factors were found.

[Carcinoma in situ of the breast: 10 cases]. / Le carcinome in situ du sein: 10 cas.

The authors have reviewed 10 cases of carcinoma in situ of the breast, which they were able to isolate from 180 invasive cancers of the breast that were operated on during the same period of time. (These rare lesions occasionally occur at the same time as an invasive cancer; then they increase the risk of local recurrences). When they occur by themselves in situ cancers of the breast tend to be duct cancers or lobular cancers. Duct lesions are more common. They do, in a significant proportion of cases, become invasive. The authors point out the value of more mammographies being taken to diagnose these duct carcinomas in the breast in order to work out the correct treatment. They think it is important to delay making a decision as to the surgical procedure to be adopted until the report on the histology of the specimen taken for biopsy has been received. For a long time total mastectomy was the only treatment: the results were too good... (some patients were overtreated). The present tendency is to limit the operation (to segmental excision) in cases where the lesion is limited. Mastectomy is reserved for cases that are spread out. Carrying out irradiation after widespread segmental excision increases the risks of local recurrences. Clearing the whole area has only to be considered when there are widespread forms of the condition. Lobular cancers in situ are marker lesions and have to be followed up very carefully indeed. The authors illustrate their article with some anatomopathological specimens and mammographies that they have come across.

[Tubo-ovarian actinomycosis, intrauterine device and chemotherapy for breast cancer]. / Actinomycose tubo-ovarienne, stérilet et chimothérapie pour cancer du sein.

Tubo-ovarian actinomycosis was observed after chemotherapy for breast cancer in a patient with an intrauterine device. Does chemotherapy increase the risk? How can it be prevented?

[Malignant cutaneous hemangioendothelioma. Clinicopathological and ultrastructural aspects (author's transl)]. / Hémangio-endothélio-sarcomes cutanés. Aspects anatomo-cliniques et ultrastructuraux.

Malignant cutaneous angio-endothelioma are tumors which develop from vascular endothelium. This is a study of one case along with the data compiled from 118 previously published cases. Clinically, this rare tumor is chiefly seen before the age of twenty of after sixty. Its appearance is polymorphic and often misleading. The clinical course of this disease in adults is always fatal, however, in children 20 p. 100 of the cases are reported curable. The histological picture shows a polymorphic pattern associated with vascular channels anastomosing in a plexiform network with large lacunae surrounded by papillary vegetations and plaques of fusiform, sarcomatoid cells as well as areas of angioblastic secretory cells. The ultrastructural study confirms that the tumor originates from endothelial cells which have lost their capacity to cover the capillary walls.

[Sirenomelia and multicystic renal dysplasia. Apropos of 2 cases]. / Sirénomélie et dysplasie rénale multikystique. A propos de deux observations.

Two cases of sirenomelia with multicystic renal dysplasia (Potter's type II A) are reported. One case was discovered on fetal ultrasonography. Multicystic renal dysplasia in sirenomelia is an additional plea for a primitive mesoblastic defect in the caudal regression syndrome.

[Anatomo-clinical features of Castleman tumour. Report of a case with a membrano-proliferative glomerulonephritis (author's transl)]. / Aspects anatomo-cliniques de la pseudo-tumeur de Castleman. Revue de la littérature à propos d'une observation avec glomérulopathie membrano-proliférative.

A case of retro-peritoneal lymphoid angio-follicular hyperplasia or Castleman's pseudo-tumour with normochromic hormocytic anemia and membrano-proliferative glomerulonephritis is reported in a 53 year-old man. Since the first description by Castleman in 1954, 259 other cases have been published. The anatomo-clinical study of our observation and of 198 cases collected in the literature bears out 3 types of clinical forms, according to the location of the pseudo-tumour: mediastinal, superficial and abdominal. The first ones generally asymptomatic are the most frequent whereas the last ones, less common, are often associated with biological abnormalities. In spite of these differences such neoformations present the same morphologic features. They are round or ovoid and well delimited by a capsule. Under this capsule a lymphoid vascular tissue surrounds follicules with central capillaries or arterioles. When plasma cells infiltrate the stroma, perturbations such as anemia or hypoalbunemia appear. Besides a nephrotic syndrome has been discovered in our observation and in that of Humpherys. In all the cases the removal of the pseudo-tumour induces the retrogression of the biological abnormalities and of the nephrotic syndrome. Neither a recidive nor another location of the disease are noticed later on. This evolution is probably the fact of a benign hyperplatic process which grows after an antigenic stimulation.

[Neonatal hepatitis with alpha-1-antitrypsin deficit. Apropos of a personal case]. / Hépatite néonatale avec déficit en alpha-1-antitrypsine. A propos d'une observation personnelle

The authors report a case of neonatal hepatitis with alpha-1-antitrypsin occuring in a child of ZZ phenotype. The anatomopathological study carried out on two liver biopsies showed changes of common cholestatic hepatitis developing into cirrhosis, as well as intrahepatocytary globulins. Moreover, these globulins, P.A.S. positive after treatment by alphaamylase, fix an antialpha-1-antitrypsine antiserum. Ultrastructural analysis shows them to be masses of amorphous material, feebly osmiophilic, outlined by a unitary membrane the moniliform aspect of which recalls the ergastoplasmic membrane. These findings are identical to those already made in cases of cirrhogenous neonatal hepatitis by alpha-1-antitrypsine deficit reported in the literature. They point out the irreversibility of the affection which, after a stage of cholestatic hepatitis with or without inflammatory portal fibrosis, develops into cirrhosis. At this stage cholestasis has regressed or disappeared whereas portal sclerosis, often infiltrated with free elements, surrounds hepatic lobules and biliary neocanaliculi. But the globulins are still present and appear to be the specific feature of this deficit. By their ultrastructural and immuno-histochemical features, these globulins would represent a form of accumulation of alpha-1-antitrypsin in the hepatocytes which normally carry out the synthesis of this antienzyme. Accumulation in the hepatocytes proves excretory disturbance of hypothetical mechanism: structural anomaly, changes in the permeability of the membrane. Its role in the occurrence of hepatitis or cirrhosis lesions is still to be demonstrated but one may think that it consists in absence of inhibition of the enzymatic factors discharged during agressions.

[Bilateral breast localization of Kahler's disease: cytological and histological aspects]. / Localisation mammaire bilatérale d'une maladie de Kahler: aspects cytologiques et histologiques.

Breast localisation of multiple myeloma is quite unfrequent. In one case of a 69 years old man, the diagnosis was suspected by a cytological aspiration biopsy of the lesion, and later confirmed by histological and biological investigations. The analysis of the literature has enabled the authors to gather 7 other cases and to describe the characters of this peculiar tumoral localisation. The breast involvement is mostly observed in women. The average is 38 years old. Only 2 cases deal with men, both treated with estrogens. The lesion is usually bilateral and nearly always associated with other visceral and bone localisations. It is not modified by chemiotherapy and appears shortly before death.

[Sinus histiocytosis with massive lymphadenopathy: a new case in an adult with anti factor VIII antibody (author's transl)]. / Maladie de Rosaï: forme grave chez un adulte avec présence d'un anticorps antifacteur VIII.

The case history of a 71 year old french man with a lethal sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman's Syndrome) is reported. The histological features were typical but the clinical course was remarkable because of the predominant expression was a life threatening hemorrhagic disorder closely linked to a circulating anticoagulant (anti-factor VIII). The bringing to light in a picture of hyperimmunity of a self antibody and a high anti-EBV titer suggest on lymphocyte T deficit could be important in the development of the sinus histiocytosis.

[Mucinous adenocarcinomas of the scalp. Anatomo-clinical, histochemical and ultrastructural study of a case and a review of the literature]. / L'adénocarcinome mucineux du cuir chevelu. Etude anatomo-clinique, histochimique et ultra-structurale d'un cas et revue de la littérature.

The third recurrence of a primary mucinous adenocarcinoma of the right parietal scalp was studied by histochemistry and electron microscopy. Histochemically the mucin produced by this neoplasm was of the sialomucine type. Electron microscopy revealed a high differentiated tumour whose cells looked like eccrine mucinous ones. The natural history of this carcinoma is similar to that of 33 ones already published. The tumours, round and elevated, grow in 75 p. 100 of the cases in patients over 50 years of age. They are located on the face or the scalp and more rarely on the trunk. Such tumours are frequently misdiagnosed as carcinomas. But the knowledge of thier morphological and histochemical features should lead to the right diagnosis all the more since the various biological and radiological investigations do not discover other primary neoplasm. Besides if these neoplasms are not metastases they are quite different from the other sweat gland carcinomas. They have a better prognosis than these tumours. However they give local recurrences, lymph node or subcutaneous metastases and a fatal issue in 6 p. 100 of the cases.