Perianal Paget's disease: report of five cases

Paget's disease of the anus is a rare perianal disorder. The condition is often associated with underlying invasive carcinoma. The prognosis is poor when rectal adenocarcinoma is present. Five own cases of perianal Paget's disease are presented. In two of our cases an underlying adenocarcinoma was found in the anorectum. Adenocarcinoma is sweat gland ducts was found in one case. One patient developed an adenocarcinoma in the anorectal junction four years after the Paget diagnose. In only one of our cases no underlying adenocarcinoma was found.

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Résumé La maladie de Paget de l'anus est une lésion péri-anale rare. Cet état est souvent associé avec un cancer invasif sous-jacent. Le pronostic est mauvais lorsqu'un cancer du rectum est présent. 5 cas personnels de maladie de Paget péri-anale sont présentés. Dans deux de ces cas un cancer sous-jacent a été trouvé dans l'ano-rectum. Un cancer des canaux glandulaires sudoripares existait chez un malade. Un patient a développé un adénocarcinome de la jonction ano-rectale 4 ans après le diagnostic de maladie de Paget. Chez un seul de nos cas seulement il n'existait pas d'adénocarcinome sous-jacent.

     

The glucagonoma syndrome. 3 new cases]

We report three new cases of glucagonoma revealed, 6 to 12 months after its onset, by non-specific and misleading skin lesions associated in all 3 cases with diabetes mellitus, severe deterioration of the general condition and hyperglucagonaemia. Non-invasive methods, such as ultrasonography and computerized tomography (CT), are most helpful to locate the pancreatic tumour. Phlebography with tiered venous blood sampling is useful in difficult cases. A false positive result has been recorded with arteriography. Ultrasonography and CT have yielded two false negative results. The alpha-chain of the chorionic gonadotrophin hormone has limited value in the diagnosis of malignant glucagonoma. Treatment is surgical, but despite it, the prognosis is severe (two of our three patients died), due to the risk of thromboembolism, to cachexia and to metastases that are frequent at the time of diagnosis.     

Perianal disease of tuberculous origin

PURPOSE: A case of anal tuberculosis in an otherwise asymptomatic patient with bleeding anal ulcers is presented. The clinical features of this entity and the problems in differential diagnosis between anal infectious vs. inflammatory diseases are discussed. METHODS: The management and outcome of the case of an adult patient who presented with perianal ulcers is described. RESULTS: On a three-drug antituberculous regimen, symptoms abated, radiographic infiltrates improved, and perianal ulcers healed. CONCLUSION: Anal tuberculosis is an extremely rare disease. A tuberculous origin must be considered when the cause of perianal ulcers is unclear to avoid undesirable delays in the diagnosis and treatment of this disease.     

Bradyarrhythmias secondary to the use of ophthalmic timolol. A report of 3 cases]

Three cases of symptomatic bradycardia due to topical ocular timolol administration are reported. Two patients had syncope related to atrioventricular block, and the other one complained of dizziness due to sinus bradycardia. Heart disease was not present in any case, although a right bundle branch block was observed in one patients. A normal sinus rhythm resumed in all patients after discontinuation of timolol.     

Nonamyloid light-chain deposit disease. A report of 2 cases]

Two cases of non-amyloid light chain deposit disease (EDCL) which were diagnosed by liver biopsy are presented, and the recent literature is reviewed. This disease is a dysproteinemia which shares numerous clinical and pathological features with amyloidosis. A commentary is made on the differential diagnosis between both diseases. This disease must be suspected when the conventional histochemical and immunohistochemical stains for amyloid are negative. Immunohistochemical assays for kappa and lambda chains should then be performed.     

Cardiac involvement in pheochromocytoma. A report of 6 cases]

Pheochromocytoma is a rare case of sustained hypertension or hypertensive crisis: sometimes it may occur with several aspecific symptoms or hypotension. The literature reports frequent myocardial involvement in patients affected by pheochromocytoma. Adrenergic hormone effects are responsible for a functional coronary insufficiency which causes myocardial damage. The cardiac involvement may appear with symptoms of different severity that sometimes represent the only symptoms of the neoplasia. Clinical and instrumental cardiac alterations observed in 6 patients suffering from pheochromocytoma are reported. Electrocardiographic abnormalities were found in 5 out of 6 patients. Inferior myocardial infarct was present in one case; in two patients electrocardiogram showed left ventricular hypertrophy and negative T wave; frequent ventricular extrasystoles in one case and complete atrio-ventricular block in another were found during the hypertensive crisis; in one patient the electrocardiogram showed STT changes and in the last one, left ventricular hypertrophy, more evident in the interventricular septum, was present at the echocardiogram. All subjects underwent surgical treatment after a period of drug therapy. To date surgery is the only possibility to eliminate myocardial malignant effects of catecholamines.     

Benign metastasizing pulmonary leiomyomatosis. A report of 3 cases

The benign metastasizing leiomyoma is an uncommon variety of leiomyoma, tumor derived from smooth muscular tissue. The benign metastasizing leiomyoma affects a middle age women, with antecedents of uterine leiomyoma, the pulmonary lesions appeared as a multiple nodules, without systemic affectation. We present three cases of benign metastasizing leiomyoma that de diagnosis was made for biopsy by thoracotomy; and in one case the markers from estrogens' receivers were positive.     

Mycetoma due to Actinomadura madurae. A report of 2 cases]

Actinomadura madurae mycetoma was diagnosed in two patients by the dermatologic outpatient clinic of the Clementino Fraga Filho University Hospital (UFRJ) and the Antonio Pedro University Hospital (UFF). The first case was a 27-year-old Negro male from the outskirts of Rio de Janeiro, who was injured prior to the onset of the disease in 1988. The affected left foot showed swelling, nodules, sinus tracts, purulent discharge containing grains, and serious bone involvement with lytic lesions. Histological examination showed deeply basophilic stained grains with typical wide-fringed borders. The mycological examination revealed an actinomycete identified as A. madurae by culture. Oral tetracycline for six months did not improve the clinical-radiological picture and the patient was submitted to amputation. The second case was a 70-year-old white male, with previous injury in 1974, which occurred in Pernambuco State. Enlargement of the right foot draining sinuses formation with discharge of pus and grains. In tissue sections the grains were large and surrounded by amorphous eosinophilic clubs radially oriented. There was discrete improvement by tetracycline and sulfonamide. No follow-up.     

Rectal leiomyosarcoma. 4 new cases]

We report four cases of leiomyosarcoma of the rectum suspected by endoscopic ultrasonography. Three patients were treated by local excision and one by abdominoperineal resection. An excision of the mass via a Kraske's approach was used. Leiomyosarcoma confined to the rectum wall can be treated by local excision. Endosonography can provide exact estimation of the lesion and is of great value in selecting the appropriate treatment. The treatment is surgical excision with wide margins. The histological stage and the presence or absence of metastases determine the therapeutic. Two patients in our series underwent radiation therapy. Chemotherapeutic agents including doxorubicin have had beneficial effect on recurrence or survival, only for higher grade sarcomas.     

Anemia: monosymptomatic celiac disease. A report of 3 cases

Patients with monosymptomatic celiac disease (CD) can escape diagnosis for a long period. Anemia is a common finding in CD, although anemia as the sole symptom is relatively unknown. We report on three patients who presented with iron deficiency anemia and no other symptom, in whom CD was considered and diagnosed several to many years after the anemia was documented.     

A new observation in the clinical spectrums of erythroleukemia. A report of 46 cases.

Erythroleukemia is a disease manifested by an abnormal proliferation of erythroid and myeloid precursors, generally consisting of a primary erythroid phase (chronic erythemic myelosis), a transition phase involving erythroid and myeloid precursors (erythroleukemia) and, finally, the purely myeloblastic (acute myeloblastic leukemia) phase. The experience at Memorial Sloan-Kettering Cancer Center is reported. Presenting signs and symptoms are consistent with prior reports. The chemotherapy results in the past have been poor; because of the poor results, chemotherapy is started only if one of the following criteria are present: (1) frequent transfusion requirements; (2) rapidly increasing peripheral white blood cell count or percentage of leukemic blast forms; (3) frequent recurrent infectious and/or hemorrhagic complications. A hitherto unrecognized association of erythroleukemia and symptomatic rheumatic disease and numerous immunologic abberations are reported. The symptoms related to this rheumatic disorder do not seem to be relieved by therapy directed at the leukemic process, but rather by the use of simple anti-inflammatory agents.     

Black-grain eumycetoma due to Madurella grisea. A report of 2 cases]

Two cases of black grains eumycotic mycetoma, occurring on a foot, are reported. Both proceeded from the state of Bahia (Brazil), and in both the etiologic agent was Madurella grisea Mackinnon et al., 1949. The grains structure as well as the micromorphologic characteristics of the fungus in saprophytic life were studied. It is the author's belief that these observations correspond to the 7th and 8th cases reported in the Brazilian medical literature. The authors do consider the following Madurella species as nomen dubium or nomina confusa: M. ramiroi, M. oswaldoi, M. bovoi, M. tozeuri, M. mansonii, M. brumpti, M. reynieri, M. americana, M. lackawanna e M. ikedae and the same for Rubromadurella mycetomi. The only valid species must be Madurella mycetomatis McGinnis, 1980 (= Madurella mycetomi Brumpt, 1905) and Madurella grisea Mackinnon et al., 1949. Treatment with itraconazole in both reported cases, for a 3 month duration, did not produce any regression of the lesions, the clinical improvement being meager.     

Non-healing iatrogenic gastric ulcers after endoscopic mucosal resection for gastric epithelial neoplasia: report of two cases]

Endoscopic mucosal resection (EMR) is widely accepted as a standard treatment for early gastric cancer or gastric adenoma. However, EMR inevitably results in the formation of large iatrogenic ulcer at the resected area. Although the characteristics of EMR-induced ulceration are not fully understood, this type of ulcer is thought to heal faster and to recur less often than non-iatrogenic gastric ulcer. Current available evidences have suggested that EMR-induced ulcers heal within 2-3 months. Herein, we report two cases of non-healing persistent gastric ulcers after EMR. One is a case of gastric carcinoma which developed at the same site of previous EMR site for the low grade dysplasia. The other is a case in which persistent EMR-induced ulcer was healed in the long run after Helicobacter pylori eradication therapy.     

Uninhibited anal sphincter relaxation syndrome. A new syndrome with report of four cases.

I report a new syndrome, in four patients, all male. Patients ranged from 36 to 43 years of age. The main complaint was fecal urgency with occasional fecal soiling. Physical examination as well as pressure and EMG studies of anal sphincters and levator ani muscle were all normal. The only positive finding was an abnormal rectoinhibitory reflex. The external anal sphincter did not contract either reflexively or voluntarily on rectal distension, leaving the relaxing internal sphincter unprotected and uncontrolled. The patients could not oppose the urge to defecate if conditions are inopportune, with resulting urgency and occasional fecal soiling. The cause is unknown, but biofeedback effected improvement in all four cases.