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.     

Bullous sarcoidosis: a report of three cases

Three cases of pulmonary sarcoidosis presented as bullous emphysema with severe airflow obstruction, and the diagnosis of sarcoidosis was unsuspected for at least 2 years. Potential mechanisms of bullous emphysema from sarcoidosis are discussed. The physician should suspect sarcoidosis as the cause of bullous emphysema when young patients who have smoked relatively few pack-years present with emphysema or severe airflow obstruction. Additional clues are the presence of mediastinal adenopathy on a chest radiograph or a CT scan and a history consistent with extrapulmonary sarcoidosis.     

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.     

Septate uterus with cervical duplication and longitudinal vaginal septum: a report of three new cases

The first case of a rare mullerian anomaly characterized by the presence of a complete uterine septum with duplication of the cervix and a longitudinal vaginal septum has been reported very recently. We present here three new cases of such an anomaly in an attempt to alert gynaecologists to the possible occurrence of such a malformation. The cases challenge the classical views of unidirectional (caudad to cranial) mullerian development and support the alternative embryologic hypothesis of Muller et al. according to which fusion and resorption begins at the isthmus and proceeds simultaneously in both the cranial and caudal directions.     

The Marcus Gunn phenomenon: discussion and report of three cases

The Marcus Gunn phenomenon and Marin Amat syndrome (inverted Marcus Gunn phenomenon) are discussed, along with their associated features. Two patients exhibiting the Marcus Gunn phenomenon only and one patient exhibiting both the Marcus Gunn phenomenon and Marin Amat syndrome are described.     

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.     

Congenital dyserythropoietic anaemias: clinical features, haematological morphology and new biochemical data

Three types of congenital dyserythropoietic anaemia (CDA) were originally identified on the basis of the pattern of dysplastic changes in the erythroblasts and the results of the acidified serum lysis test (Ham test). These were designated CDA types I, II and III. Several other types have been described subsequently and new forms continue to be reported. Some patients with CDA develop iron overload even without repeated blood transfusion and may present with the complications of severe iron overload. Dysmorphic features are seen in some cases, especially of CDA type I. In CDA type II, incomplete processing of N-linked oligosaccharides leads to a marked reduction of polylactosamines associated with band 3 of the red cell membrane. A few cases of CDA type III develop lymphoid neoplasms. Some of the Swedish cases of CDA type III have developed a retinal abnormality characterized by angioid streaks and macular degeneration. The chromosomal localizations of the disease gene in CDA types I and II and in the Swedish family with CDA type III are now known, but the identities of the mutant genes are still unknown. Cases of CDA type I have shown a partial haematological response to interferon-alpha, however the biochemical basis of this response is unclear. An important step in the diagnosis of sporadic cases of CDA is the exclusion of known causes of acquired dyserythropoiesis.     

Rapidly growing histologically benign meningiomas: cell kinetic and deoxyribonucleic acid ploidy features: report of three cases

We present three cases of histologically benign meningiomas with a rapid and known time course to the development of symptoms. Tumor doubling time calculated from sequential computed tomographic scans and computer-assisted image analysis of proliferating cell nuclear antigen reactivity suggested rapid growth. Feulgen staining indicated deoxyribonucleic acid aneuploidy. Tests for progesterone and estrogen receptor immunoreactivity were negative. These cases are noteworthy for their uncharacteristically rapid growth in the absence of histological evidence of atypia.     

Reading-induced epilepsy: three new cases

INTRODUCTION: Primary epilepsy of reading is a rare syndrome in which patients present with mandibular myoclonia when reading texts. Seizures are also frequently provoked by other stimuli. Occasionally they may be followed by generalized tonic-clonic seizures. CLINICAL CASES: We present the clinical features and electroencephalographs of three patients with epilepsy of reading, one of them also had seizures when playing chess and after doing arithmetic and the third after reading music. Treatment with valproic acid and clonazepam completely controlled the seizures in all three patients. In two cases we saw epileptiform activity on EEG whilst they were reading, although the basal EEG was normal. CONCLUSIONS: We believe that epilepsy of reading is under-diagnosed and emphasize the importance of careful questioning of epileptic patients as to factors which may precipitate the seizures. In view of the social problems which may arise, early recognition of the syndrome is important for the patient.     

The effect of muscle relaxants on cricothyroid muscle: a report of three cases

PURPOSE: To determine the frequency of the computed tomographic (CT) pattern of nonaneurysmal perimesencephalic subarachnoid hemorrhage in the setting of ruptured posterior fossa aneurysms. MATERIALS AND METHODS: Four neuroradiologists independently and retrospectively reviewed cranial CT scans of 169 patients with ruptured vertebrobasilar aneurysms (44 cases of posteroinferior cerebellar artery aneurysm, 20 cases of superior cerebellar artery aneurysm, and 105 cases of basilar-tip aneurysm). RESULTS: The mean frequency of nonaneurysmal perimesencephalic pattern of subarachnoid hemorrhage in ruptured vertebrobasilar aneurysms was 7.1% (48 of 676 readings) among the four readers. The mean frequency of aneurysms with the pattern of hemorrhage for each location was as follows: basilar tip, 9.8% (41 of 420 readings); superior cerebellar artery, 5.0% (four of 80 readings); and posteroinferior cerebellar artery, 0%. In 75% (six of eight) of the cases in which the CT pattern of hemorrhage was deemed compatible by all readers with nonaneurysmal hemorrhage, the clinical presentation was mild. CONCLUSION: Because ruptured posterior fossa aneurysms manifest with the nonaneurysmal pattern of hemorrhage in approximately 10% of cases, a high degree of suspicion should be maintained even if the pattern of subarachnoid hemorrhage suggests a nonaneurysmal origin and clinical symptoms are mild.     

Capture myopathy in Elk in Alberta, Canada: a report of three cases

Acute capture myopathy was diagnosed in 3 elk (Cervus canadensis). In 2 of the elk, hindlimb lameness developed within 10 days of capture. Clinical signs were not observed in the third elk, which was found dead 7 days after its capture. Elks 1 and 2 were euthanatized and all 3 animals were necropsied. In elks 1 and 2, rupture of the semi-membranosus muscle was associated with massive hemorrhage and swelling. Microscopic findings included hemorrhage, edema, myofibril fragmentation, and evidence of attempted muscular regeneration. In elk 3, several of the large muscles of the hindlimbs as well as the biceps brachii muscles of the forelimbs appeared pale, dry, and friable. Except for lack of hemorrhage and edema, microscopic findings in this elk were similar to those of elks 1 and 2.     

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.     

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.     

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.