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An unusual case is reported in which sarcoidosis presented originally as a cheek mass unrelated to the parotid or submandibular glands. The clinical findings were highly suggestive of a neoplastic process. A general review of sarcoidosis follows the case report.
Plasma cell gingivitis is a disorder first described in the 1960s and was believed to be caused by an allergic reaction to flavored chewing gum and toothpaste. The lesion was believed to have been largely eliminated by removing the allergens from the products. We report two additional cases, not related to a known allergen. One patient was allergy tested thoroughly and given a strict elimination diet without resolution. Immunofluorescence study suggests a reactive rather than a neoplastic process.
Infantile myofibromatosis (IMF) is a benign localized (solitary) or generalized (multicentric) proliferation of fibroblastic tissue occurring exclusively in infants and children. Three cases of solitary IMF involving the posterior region of the mandible of young children are reported. These lesions manifested clinically as asymptomatic bony expansion and roentgenographically as circumscribed lytic areas. Microscopically these tumors showed a distinct zoning phenomenon of curving bundles or intertwining fascicles of plump, spindle-shaped cells at the periphery and solid sheets of less differentiated round cells in the center. Positive immunostaining for vimentin and actin, with the lack of desmin and S-100 protein reactivity, confirmed their myofibroblastic nature of these cells and supported the diagnosis of IMF. All three lesions were treated by curettage and the follow-up showed no incidence of recurrence or any other complications. As we demonstrate in these case reports, IMF should be included in the differential diagnosis of spindle cell neoplastic processes in children.
We report a fortuitous occurrence of a small bowel leiomyosarcoma infected with Schistosoma mekongi in an elderly patient from Kampuchea. S mekongi is endemic in countries along the lower Mekong River basin (Laos, Thailand, and Kampuchea), where its intermediate snail hosts (Lithoglyphopsis aperta) are found. The diagnosis was made by examining the numerous ova that were embedded in the tumor and along the small bowel wall. The ova with their small lateral spines are easily confused with those of Schistosoma japonicum, but are consistently smaller and round. The patient presented with lower gastrointestinal bleeding secondary to tumor invasion of the small bowel rather than from heavy parasitic infection. Because of the spindle-cell morphology of the tumor, an exuberant fibroblastic host reaction to the ova was considered during frozen section examination. Unlike the more common species of schistosoma (eg, Schistosoma mansoni and Schistosoma haematobium) that are associated with the development of malignant neoplasms, we believe our case represents a chance finding and that the possible role of S mekongi infection in the neoplastic process is unlikely.
The effect of various immunosuppressive treatments on mean life-span and disease incidence have been studied. Significant life shortening was seen only in mice which recieved X-irradiation early in life and can be ascribed primarily to an increased incidence of certain malignancies. Marginal life shortening was seen in cyclophosphamide-treated animals, however, survival patterns between those and control animals did not differ until 30 months of age and the magnitude of life-shortening never approached that seen in X-irradiated animals. Thymectomy, splenectomy or cortisone treatment did not alter survival. All immunosuppressive treatments enhanced mortality due to non-neoplastic diseases, however, only a small percentage of animals die with these disease entities. With the exception of cortisone all immunosuppressive treatments increased the incidence of neoplastic disease. However, their effects on various neoplastic processes were variable and unpredictable. Four primary patterns in terms of relative immune competence, disease incidence and life expectancy were seen. Thus, immunodepression may of may not correlate with increased disease incidence, which in turn may or may not have a life-shortening effect. These findings are discussed in terms of the marked reduction of both humoral and cell-mediated immunity normally seen in aged mice and the significance of postulated immune surveillance mechanisms to survival.
Fifteen patients with thoracic spinal cord compression from metastatic neoplastic processes were managed by spinal canal decompression via a modified costotransversectomy approach. Ten of the patients also underwent sequential posterior stabilization with Luque or Harrington instrumentation based upon proximity of the lesion to the thoracolumbar junction, prognosis for regaining or maintaining ambulatory ability, and additional spinal stability considerations. A modified lateral decubitus position with the scapula falling away from the side of exposure was used for T1-5 segment lesions, and a prone position was used for the (T-6)-(T-12) segment. Adequate decompression of the spinal canal was achieved in all cases. All patients who were ambulating preoperatively maintained ambulatory ability, and pain and/or further neurological improvement as well occurred in 75%.
This report details the first published case of a urethral polyp presenting as a prolapse through the proximally displaced meatus of a child with hypospadias. Urethral obstruction, a symptom common to other reported urethral polyps, was absent. The congenital nature suggests a benign neoplastic process as the etiologic factor.
The incorporation of single and combination chemotherapy into the therapeutic regimen for some advanced genitourinary tumors has greatly added to the myriad of medical and psychologic problems already present in these patients. Working closely with a urologist, a nurse clinician can be of significant help in the total care of this group of patients. Due to the seriousness and chronicity of the neoplastic process, the emotional and social impact as well as physical factors affect all phases of the patient's life, as well as the lives of family members. The incorporation of traditional nursing values into the medical education of nurse clinicians helps to prepare them to deal with these problems, and their participation serves to make the medical team more complete.
The diagnosis of renal abscess is still a challenging problem. Early manifestations are usually nonspecific and nonlocalizing. Clinical picture, laboratory data, and intravenous pyelogram do not always differentiate between inflammatory lesions and neoplastic processes. Two cases with bilateral, metachronous renal abscesses are presented. The use of 111Indium scan is probably the major recent advance used for unmasking this inflammatory lesion. Sonography, computerized tomography scan, and even arteriography are sometimes necessary to establish reliable preoperative diagnosis. The diagnostic strategy for a systematic approach to the lesion is outlined.
Scrotal abnormalities are difficult to assess using clinical criteria alone. Ultrasound provides an accurate means of demonstrating the scrotal contents so that appropriate therapy may be instituted. In a retrospective study, 119 ultrasound examinations of 96 patients (aged 4 days to 23 years) have been compared with the clinical diagnosis, surgical/pathologic findings, and other imaging modalities. The gamut of disease identified included congenital anomalies, neoplasm, trauma, torsion, varicocele, hydrocele, epididymo-orchitis, epididymal cyst/spermatocele, and post-radiation fibrosis. The ultrasound findings correlated well in 93/96 patients. In inconclusive cases, sequential imaging helps differentiate traumatic and inflammatory lesions from neoplastic processes.
(1) Histiocytosis-X can manifest itself in virtually every organ, but in gynecology it is an absolute curiosity. (2) Differential diagnosis must exclude specific and nonspecific ulcerations and granulations such as syphilis, tuberculosis, Boeck's disease, and also neoplastic processes like lymphomas, sarcomas, carcinomas, and malignant diseases of the hemopoietic system. (3) The diagnosis by light microscopy alone, as in our case, may be insufficient; therefore, electron microscopy should be used. As soon as the diagnosis is confirmed histologically, an extensive examination of all organs is necessary in order to establish an exact prognosis and an optimal plan of therapy. (4) Because of the unknown etiology of histiocytosis-X, a causal treatment is not yet possible. In spite of this, with a symptomatic, individualized therapy by means of excision, low-dose irradiation and cytotoxic agents a 5-year survival of 90% was obtained for the patients. (5) Because of its rarity and multidisciplinary character, histiocytosis-X is a challenge to interdisciplinary and interregional cooperation. Though not being a malignoma in the strict sense, diagnosis, therapy, and in part prognosis are not essentially different from a malignant disease.
Simple goitre is defined as an enlargement of the thyroid gland that is not the result of an inflammatory or neoplastic process and is not associated with thyrotoxicosis or myxoedema; the cause is unknown in most cases. Structural or regulatory defects in the proteins involved in thyroid metabolism might be involved in the functional abnormality that brings about the disorder. We have found a mutation within exon 10 of the thyroglobulin gene in 25 of 56 members of three families affected by simple goitre; 14 of the gene carriers had the disorder. DNA sequencing showed a mis-sense mutation within thyroglobulin gene exon 10, resulting in a glutamine to histidine substitution. Thus, some cases of non-endemic simple goitre are associated with a mutation at the thyroglobulin locus.
Acute promyelocytic leukemia (APL) serves as a paradigm in clinical and biological leukemia research. Firstly, APL represents a model for the new therapeutic approach of differentiation therapy, taking advantage of the ability of APL cells to respond to retinoic acid treatment by terminal differentiation. Secondly, the 15;17 chromosomal translocation specific for APL leads at the molecular genetic level to a chimeric gene fusing the PML and RAR alpha genes and appears to be an instrumental, if not actually the causative event, in the neoplastic process. These unique characteristics of an otherwise rather rare disease have recently attracted intense research interest. As in other types of leukemia where continuous cell lines are powerful research tools, studies using APL-derived cell lines have contributed a large body of relevant data in efforts to unravel the pathobiology and leukemogenesis of APL. Three cell lines have been reported to be derived from patients with APL: HL-60, NB-4 and PL-21. Both HL-60 and PL-21 lack t(15;17) while NB-4 carries this cytogenetic hallmark pathognomonic for APL. Morphological and immunophenotypical examinations of the cell lines do not permit a clear assignment to any stage of myelomonocytic differentiation. Some additional data, such as expression of myeloperoxidase, monocyte-specific esterase and annexin VIII, together with the cytogenetic and molecular biological information, suggest that NB-4 is the only genuine promyelocytic leukemia cell line, whereas HL-60 may represent a discrete stage of differentiation between the late myeloblasts and the promyelocyte; PL-21 has distinct features associated with monocytic cells. These cell lines provide unique in vitro model systems for studying the cellular and molecular events involved in the proliferation and differentiation of normal and leukemic myelomonocytic cells.
Detailed clinical histories and cytogenetic investigations using short-term cultures are reported in three typical benign lipomas. Although a diploid (normal) karyotype was observed in two cases, a reciprocal chromosome translocation t(3;12)(q28;q14) was found in the third case, which was briefly reported previously. These data are discussed in light of a lipoma with similar karyotypic changes reported by Heim et al. and a similar translocation observed by us in malignant myxoid liposarcomas. The nonrandom involvement of segment 12q13-q14 in benign and malignant lipomatous tumors suggest a common basis for at least one of the possible multiple steps in the genesis of neoplastic processes.
Cytogenetic and molecular data of three patients affected by primary myelofibrosis with myeloid metaplasia (PMMM) evolving to blastic crisis are reported. The cytogenetic findings were uncommon. The first patient (female) showed an idic(X)(q13) as the sole alteration in chronic phase, with an additional r(7) in 67% of the cells of the blast crisis; the other two patients showed, in blast crisis, a partial trisomy of the long arm of chromosome 1, without translocation, as a unique structural abnormality. These findings confirm the presence of nonrandom, although nonspecific, alterations in PMMM that, in our cases, seem to be related to the multistep progression of the neoplastic process. Molecular investigations have been applied to study the genomic organization and the level of expression of genes such as bcr and calcyclin and c-fms protooncogene possibly involved in the molecular mechanisms underlying cell proliferation in hematopoietic cells. The data obtained are discussed with respect to the myeloproliferative disorder.
Abnormal thrombopoiesis has been described in acute leukemias associated with inv(3) (q21q26.2) or t(3;3)(q21;q26.2). We reviewed 13 patients seen at the Mayo Clinic since 1979 with inversions of chromosome 3 or related abnormalities; 12 acquired and one constitutional. The patient with the constitutional abnormality had an inv(3)(p21q29) and mild leukocytosis and thrombocytosis. Among the 12 patients with acquired abnormalities, five had inv(3) (q21q26.2), three had t(3;3)(q21;q26.2), one had del(3)(q12q21), one had ins(6;3) (p21;q21q26.2), one had inv(3)(p21q12), and one had r(3)(p?21q?21). Each of these patients developed acute leukemia; eight had antecedent myelodysplastic syndrome, eight presented with platelet counts greater than 100 x 10(3)/microliters, and six had atypical megakaryocytic hyperplasia. Five patients had ringed sideroblasts in their marrow, an antecedent refractory anemia with ringed sideroblasts, or erythroleukemia. Seven patients received chemotherapy but showed no response. From the time of chromosome study, the median duration of survival was 4 months. Our results suggest that 1) although abnormal megakaryocytopoiesis is observed in patients with inv(3)(q21q26.2), multiple hematopoietic lineages are also involved in the neoplastic process; 2) an antecedent myelodysplastic syndrome is common in acute leukemia with inv(3) or related abnormalities; 3) affected patients have a poor survival and are resistant to conventional chemotherapy; and 4) abnormal megakaryocytopoiesis in acute leukemia may also be associated with pericentric inversions of chromosome 3.
A girl aged 4 years 3 months with sporadic unilateral Wilms' tumor associated with Wiedemann-Beckwith syndrome, but without aniridia, was found to have a t(X;20) in the tumor cells. Karyotypes of peripheral blood of the patient and her parents were normal. This translocation was confined to the tumor and not been previously reported either in nephroblastoma or any other neoplastic processes. Although there is no microscopic deletion on chromosome 11 and catalase activity was not decreased, we cannot rule out the possibility of a point mutation or a submicroscopic deletion.
A monosomy 7 was first detected in a 6-month-old boy with a chronic myelomonocytic leukemia. After etoposide treatment, relapse occurred after 29 months, with transformation of the disease into an acute myeloblastic leukemia. After bone marrow transplantations, two abnormal clones were found in marrow cells: 45,XY,-7,del(12)(p11p12)(66%), and 45,XY,-7,t(3;12)(q26;p12)(33%). Several karyotypic studies performed until the terminal phase exhibited the persistence of these two clones in the same proportion, although both independently acquired additional and often similar anomalies. The clone with t(3;12) acquired der(7),der(11),der(17),der(8),der(10),-5,-20, and the clone with del(12p), del(5q),der(4),der(8),der(10),der(17),-5,-20. The anomalies in 12p12 appear to represent an important although secondary event of the neoplastic process. The other anomalies may correspond to either those of a secondary acute nonlymphocytic leukemia, since they occurred after treatment by etoposide and alkylating agents, or to the natural evolution of myelomonocytic leukemia.