Metastasizing pleomorphic adenoma: a report of three cases

BACKGROUND: This study evaluates the diagnosis and treatment of women with pathologic nipple discharge caused by ductal carcinoma in situ (DCIS). METHODS: Women with unilateral spontaneous bloody, serous, or brown nipple discharge who presented between January 1, 1988 and August 1, 1996 were identified by retrospective chart review. Women with nonspontaneous, physiologic discharge were excluded. RESULTS: Two hundred seventy-seven women with a mean age of 59.5 years (range, 24 to 88 years) underwent duct exploration and biopsy for pathologic discharge, with 43 (15.5%) found to have DCIS. The discharge was bloody in 29, clear in eight, and brown in six women. Seven of 12 (58%) women with an associated breast mass were found to have a microinvasive component with the DCIS. Discharge cytology showed malignant cells in only two of 12 (16%) women examined. A ductogram was performed on 20 women, with filling defects seen in 10, ectasia in 3, narrowing in 4, and normal ducts in 3. The DCIS included 17 (40%) specimens with cribriform pattern, 17 (40%) micropapillary, 8 (18%) comedo, and 2 (2%) solid. Twelve microinvasive cancers were found in combination with DCIS. After duct exploration, 37 (86%) patients were found to have extensive or multifocal DCIS to the margin, or both, with 32 (74%) patients requiring mastectomy to achieve free surgical margins. There was residual disease in 27 of 32 (84%) mastectomy specimens after initial biopsy. Breast conservation was possible in only 11 (26%) women. Forty of 43 (93%) are disease-free with a median follow-up of 37 months. CONCLUSION: Women presenting with pathologic nipple discharge require duct exploration regardless of cytologic or radiologic findings. When discharge is the result of DCIS, extensiveness of disease in relation to central location and intraductal spread may preclude breast conservation in as many as 27 of 43 (63%) cases.     

Wandering spleen in childhood: a report of three cases

Wandering spleen is a rare cause of abdominal pain in children, and an accurate diagnosis is seldom made preoperatively. A splenectomy is the treatment of choice in cases of splenic torsion and infarction, while in patients with chronic symptoms splenopexy may also be attempted. We herein report three patients with wandering spleen, of whom two presented with acute torsion of the splenic pedicle and one demonstrated an asymptomatic mobile abdominal mass. In the first case splenopexy was attempted, but during follow-up the spleen was found to have undergone atrophy. The presentation, diagnostic procedures, and treatment modalities in pediatric wandering spleen are reviewed.     

Colorectal schistosomiasis: report of three cases

The facility for mass movement of segments of our world populations creates the need for physicians to recognize and manage diseases not seen in native patients. Such a need has occurred in New York City, where schistosomiasis, with its protean manifestations has been seen with increasing frequency. The cases of three patients who had different clinical manifestations of infestation by S. mansoni are presented. Clinical, radiologic, and pathologic features are discussed. To avoid delay in treatment, physicians must consider the possibility of this disorder in cases of patients from areas of endemic schistosomiasis.     

Lingual thyroid: report of three cases

Lingual thyroid is a rare developmental disorder and is more frequent in women. The pathogenesis is unclear but may be related to the presence of maternal blocking autoantibodies against the thyroid. Treatment of this disorder includes the use of levothyroxine in order to correct the hypothyroidism, which is very frequent and to induce the shrinkage of the gland. When symptoms of obstruction or bleeding appear, ablative therapy by means of surgery or radioiodine is warranted. We report three cases and discuss the approach to diagnosis and a strategy for management.     

Epididymal sarcoidosis: a case report]

A rare case of epididymal sarcoidosis is reported. A 52-year-old man was admitted with a painless mass in the left scrotum. An operation revealed that a 1-cm mass was located at the epididymal head and well demarcated from the testis and the surrounding tissue. Epididymectomy was performed. Histopathological diagnosis was noncaseating granulomas consistent with sarcoidosis. Systemic examination showed bilateral hilar lymphadenopathy and bilateral peripheral anterior synechiae, but these lesions were diagnosed as inactive. Serum level of angiotensin-converting enzyme was normal.     

Severe antecollis during antipsychotics treatment: a report of three cases

1. The authors report on three patients with severe antecollis that developed after chronic treatment with antipsychotics. These patients had been treated with neuroleptics for their delusions and hallucinations. Antecollis is a rare form of tardive dystonia that has drawn little attention. To our knowledge, there have been no reports on this disorder. 2. The patients developed the symptom after receiving a number of antipsychotics for 4 months to 14 years. Neither discontinuation of antipsychotics nor administration of anticholinergic agents affected the symptom. 3. Although spontaneous improvement of dystonia was obtained after several months without any specific treatment in one patient, the symptom persisted for several years in another. The last patient could not be followed after discharge from our hospital since she and her family did not come to the hospital. 4. Severe antecollis may cause disturbances in various functions, such as vision, speaking and swallowing. Forward bending of the neck may cause inspiratory obstruction. Severe antecollis may not be unusual and psychiatrists should be aware of this symptom in routine clinical practice.     

Congenital dyserythropoietic anaemia: report of three cases

Between January 1985 and June 1992, the Paediatric Department of Hospital Universiti Sains Malaysia has diagnosed congenital dyserythropoietic anaemia in three children, two of whom were siblings. The age of onset ranged from 1 to 3 years. All of them became transfusion-dependent before the age of 4 months. One of them was successfully treated with bone marrow transplantation.     

Unilateral lung hypoplasia: report of three cases

The authors describe a unique pulmangiographic picture taken 24 years after the first angiographic examination made on account of repeated haemoptysis associated with Rendu-Osler-Weber s disease. Angiography revealed enlargement of the aneurysm in the lungs and dilatation of the afferent and efferent vessel of the aneurysm. The only treatment of pulmonary arteriovenous fistulas is surgical.     

Endolymphatic sac tumors: report of three cases

OBJECTIVE AND IMPORTANCE: We present three cases of endolymphatic sac tumors and review the previously published cases. Despite frequent extension to the cerebellopontine angle, these rare tumors have only recently been recognized by neurosurgeons. CLINICAL PRESENTATION: A 26-year-old man developed a progressive hearing loss, revealing an intrapetrous retrolabyrinthine tumor on the right side. A 28-year-old woman experienced a left cerebellopontine angle syndrome, with a lytic intrapetrous mass extending into the cerebellopontine angle. A 38-year-old woman presented with an intracranial hypertension syndrome caused by a tumor of the jugular foramen. INTERVENTION: For the first and second patients, the tumors originated from the operculum of the endolymphatic sac. Total removal was achieved, via a transpetrosal approach, in these two cases. No recurrence was detected after a 20-month follow-up period. For the third patient, the tumor originated from the distal part of the sac. Recurrence was observed 8 years after subtotal removal via a retrosigmoid route. Histological analysis revealed a papillary-cystic adenocarcinomatous pattern in all cases, without features of aggressiveness. CONCLUSION: Endolymphatic sac tumors are locally invasive neoplasms characterized by bipolar intrapetrous and posterior fossa involvement. The anatomic complexity of the endolymphatic sac may explain the distinct patterns of extension of these tumors. Early radical surgery is related to good outcomes.     

Oral ulceration as a presenting sign of unknown sarcoidosis mimicking a tumour: report of 2 cases

OBJECTIVE: To test whether some genotypes for CYP2D6 or CYP2C19 could contribute to longevity, we genotyped 241 Danish nonagenarians and centenarians for CYP2D6 and CYP2C19. METHODS: For CYP2D6 we identified the alleles CYP2D6*1, CYP2D6*3 and CYP2D6*4 with allele-specific polymerase chain reaction (PCR). The CYP2D6*5 alleles were identified with a long PCR method. For CYP2C19 we identified the alleles CYP2C19*1, CYP2C19*2 and CYP2C19*3 with an oligonucleotide ligation assay. RESULTS: The four alleles for CYP2D6 did not occur in Hardy-Weinberg proportions. The frequency of poor metabolism was slightly higher (10.2%) than expected [7.7%; odds ratio (OR) = 1.36 (0.75-2.40)]. The genotypes for CYP2C19 occur in Hardy-Weinberg proportions. The frequency of poor metabolism (3.8%) was not significantly different from a young control group [3.1%; OR = 1.21 (0.26-5.75)]. CONCLUSION: CYP2D6 could play a role in human longevity due to the lack of Hardy-Weinberg proportions. If CYP2D6 only plays a role in longevity by protecting the poor metabolizers from cancer, we should expect a rise in the frequency in these genotypes in Denmark from 7.7% among young adults to 10-11% among very old people. We found a frequency of poor metabolism of 10.2% in the very old group. CYP2C19 is - due to the occurrence of Hardy-Weinberg proportions and the expected number of poor metabolizers unlikely to contribute to human longevity.     

Cytogenetic abnormalities in pediatric myelodysplastic syndrome: a report of three cases

Three consecutive cases of pediatric myelodysplastic syndrome (MDS) diagnosed over a three-year period in Queen Mary Hospital, Hong Kong, were described. Depending on the classification system used, they comprised two cases of chronic myelomonocytic leukemia (CMMoL) of which one can be reclassified as juvenile chronic myeloid leukemia (JCML) and one cases of refractory anemia with excess of blasts (RAEB) or an alternative diagnosis of atypical CML. Cytogenetic abnormalities were detected in all of them on examination of bone marrow cells. Of the two CMMoL, one had monosomy 21, whereas the other had hypodiploidy. The patient with RAEB had a complex karyotype of 46,X,del(X)(q24),t(1;7) (p22;q32),add(15)(q26)(8). The balanced translocation (1;7) seen in this patient was exceedingly rare and, to the best of our knowledge, was reported only twice in the literature. The karyotypic abnormalities that we saw in our patients were not well recognized in pediatric MDS. This report emphasizes the importance of cytogenetic study in children suspected of suffering from MDS, which remains a rare disorder of childhood, and a need to rationalize current classification schemes.     

Intranodal palisaded myofibroblastoma: report of three new cases

Intranodal palisaded myofibroblastoma is a well-defined entity in the group of spindle-cell neoplasms of lymph nodes. The authors report 3 new cases of this unusual tumor. The nature of the proliferating cells is discussed.     

Autosomal recessive omodysplasia: report of three additional cases

AIM: To determine the results of transluminal angioplasty in patients with Brescia-Cimino arteriovenous fistulae. PATIENTS AND METHODS: Thirty-one patients underwent transluminal angioplasty of 36 stenotic lesions related to Brescia-Cimino arteriovenous fistulae over a 5-year period. The lesions treated were characterized by review of pre-angioplasty fistulograms. Medical and radiological records were reviewed to assess medium-term patency of each patient's fistula. RESULTS: Angioplasty was performed successfully in 28 out of 31 patients initially (90% technical success rate). Duration of follow-up for the 31 patients ranged from 4 to 65 months (median = 34 months). At 6 months, seven patients required further surgical or endovascular intervention (18 patients remained event-free) and at 1 year, 10 patients required further endovascular or surgical intervention (14 patients remained event-free). Life-table analysis revealed primary patency rates of 77%, 64% and 39% at 6 months, 1 year and 2 years, respectively. At 6 months and 1 year, four and five patients, respectively, required surgical revision or closure of fistula. Secondary patency rates were 85%, 81% and 65% at 6 months, 1 year and 2 years, respectively. All patients with a primary patency at 2 years remained event-free during the follow-up period. CONCLUSIONS: Transluminal angioplasty is an effective treatment for stenoses developed in relation to Brescia-Cimino haemodialysis fistulae. Further endovascular procedures may be required, especially in the first 24 months, to preserve patency. These techniques extend the lifetime of fistulae, thereby preserving proximal venous access sites for future use. Our result is in broad agreement with results from other series.