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[A case of endobronchial minute leiomyoma and literature review of the 66 cases of endotracheal and endobronchial leiomyoma reported in Japan].

A case of endobronchial minute leiomyoma successfully treated by bronchoscopically directed forceps biopsy is described. A 42-year-old male with a 20 pack-year smoking history was admitted for dry cough occurring at night. Chest X-ray showed no abnormal shadows. The tumor, measuring 2 by 2 mm, was located in the right B7. The clinical characteristics of the 66 cases of endotracheal and endobronchial leiomyoma reported in Japan are also discussed. The male to female ratio of this disease was 2:3. Middle-aged people were most, commonly affected. Usually, obstructive pneumonia or atelectasis, which develops distal to the lesion, causes respiratory symptoms and chest X-ray abnormality. However, 10% of cases were asymptomatic and 30% had a negative chest X-ray. There were 7 cases of endobronchial minute leiomyoma, measuring less than 10 mm in diameter. Of these, three cases had only hemoptysis and had no chest X-ray abnormality. In such cases fiberoptic bronchoscopy is may be the only useful procedure for the diagnosis of this disease.

Adult

[Clinical x-ray diagnosis of gastric leiomyomas].

Under observation were 23 patients with leiomyomas of the stomach. Leiomyomas were more frequently observed in females, in the proximal segment these were as frequent as in the distal one, but more frequently in the posterior wall. Submucous endo gastric localization was typical for most leiomyomas. The possibilities of clinico-roentgenological diagnosis were studied. The clinical picture of leiomyomas of the stomach shows pains in the epigastric region, general disturbances, acute profuse gastric hemorrhages. The most typical roentgenological sign of gastric leiomyoma is an oval or round filling defect with even margins around 5-6 cm in size. Not infrequently there are one or several ulcerations on the leiomyoma surface like "a niche" of oval or semioval shape. The mucosa folds around the filling defect are moved apart but not destroyed. Some information on gastroscopic examination of 8 patients with gastric leiomyomas is reported.

Adult

Oral leiomyomas.

Oral leiomyomas are considered to be rare neoplasms, but they may be encountered more frequently than generally believed. Three types of leiomyomas are commonly described: solid leiomyomas, angiomyomas, and epithelioid leiomyomas. Three cases of solid leiomyoma are presented, all of which occurred in the anterior mandibular mucobuccal fold. Leiomyomas can be easily confused with other spindle-cell tumors. The necessity of using special stains, especially Mallory's phosphotungstic acid hematoxylin, is discussed.

Adult

Leiomyoma and leiomyosarcoma of the orbit.

Comparison of the clinical and pathologic features of two orbital leiomyomas, two leiomysarcomas, and one embryonal rhabdomyosarcoma showed the leiomyomas occurred in young individuals and the leiomyosarcomas in older patients. The histopathologic diagnosis rested on the intense cytoplasmic eosinophilia and nostriated longitudinal cytoplasmic filaments demonstrated by means of the trichrome stain. The leiomyosarcomas disseminated 15 months and seven years after their orbital presentations. The treatment of both leiomyoma and leiomyosarcoma is surgical. Leiomyomas are encapsulated growths that may have small satellite nodules projecting from the main tumor mass; thus, a margin of normal tissue should also be excised, lest a small lobulation be left behind to serve as the seed for a late recurrence. Once the diagnosis of leiomyosarcoma has been made, and no evidence of metastasis has been found after a thorough systemic evaluation, the orbit should be exenterated, because the tumor is unencapsulated and liable to widespread dissemination. Rhabdomyosarcoma has a much more fulminant course than leiomyosarcoma, and especially more so than that of leiomyoma of childhood. The histopathologic diagnosis of a malignant smooth muscle tumor in a child should always be questioned, since embryonal rhabdomyosarcoma is a much more likely diagnosis.

Adolescent

Genitourinary leiomyomas.

Leiomyomas may originate from any anatomic location of smooth muscle in the genitourinary system but are uncommon neoplasms. Five unusual cases of leiomyomas arising from the renal pelvis, bladder, spermatic cord, and glans penis are presented. The leiomyoma arising from the glans penis in a three-year-old boy is the first case of a leiomyoma in that location noted in the literature. A review of leiomyomas from each genitourinary structure of origin is presented.

Child, Preschool

Leiomyoma-derived growth factors for smooth muscle cells.

The presence of growth substance(s) for smooth muscle cells and fibroblasts was documented in extracts of human leiomyoma tissues (Koutsilieris et al, Am. J. Obst. Gynecol. 163: 1665-1670, 1990). In this study leiomyoma tissues (700 grs) obtained at surgery were extracted and purified using CM-sepharose, heparin-sepharose and reverse-phase high performance liquid chromatography (r-HPLC). The mouse NIH/3T3 cell fibroblasts (ATCC-CRL 1658) and the rat smooth muscle cells A10 (ATCC-CRL 1476) were used as indicator bioassay systems assessing the proliferative effect (tritiated-thymidine incorporation and cell number) of leiomyoma extracts. The profile of peptides purified by cm-sepharose, heparin-sepharose and by r-HPLC was analyzed by standard SDS gel electrophoresis. An overall 50,000 fold purification was achieved of a preferentially acting material on rat aorta smooth muscle cells (A10 cells). This material possessed mitogenic activity equivalent to epidermal growth factor (EGF) on A10 smooth muscle cells, in vitro. We believe that the final chemical definition of such leiomyoma-derived growth substance with preferential action on smooth muscle cells may elucidate the paracrine and/or autocrine interactions among myometrial cells and thus could provide new clues for the pathogenesis of uterine leiomyomas.

3T3 Cells

[Leiomyoma of the portal vein].

A case report of a 34-year-old female with leiomyoma of the portal vein is presented. The tumor was found accidentally, when laparatomy with diagnosis of cholelithiasis was performed. Total excision of the leiomyoma including a part of the anterior wall of the portal vein was necessary and could be carried out. The continuity of the portal vein could be preserved using continuous vascular suture. Microscopical examination of the tumor showed an increased proliferation rate in the leiomyoma; therefore the tumor was classified "semimalignant". A review of the literature concerning leiomyoma and leiomyosarcoma of the central and the peripheral veins showed 91 published cases and no other report of portal vein leiomyoma. The tumors of the vena cava inferior, which have been observed mostly, are listed in particular.

Adult

Mesenchymal tumors of the uterus. VI. Epithelioid smooth muscle tumors including leiomyoblastoma and clear-cell leiomyoma: a clinical and pathologic analysis of 26 cases.

Twenty-six cases of atypical smooth muscle tumors of the uterus, including leiomyoblastoma, epithelioid leiomyoma, clear-cell leiomyoma, and plexiform tumorlet, are presented. The characteristic microscopic feature serving as the basis for inclusion in this study is the rounded to polygonal shape of the majority of cells instead of the elongated blunt-ended shape of smooth muscle cells seen in the typical leiomyoma. Mixtures of epithelioid, clear-cell, and plexiform patterns occurred with sufficient frequency to indicate that such patterns are variants of a single entity. A transition to typical smooth muscle cells was found in most instances, confirming the smooth muscle origin of these tumors. Features related to a favorable prognosis include the presence of clear cells, an expansile tumor margin, extensive hyalinization, and absence of extensive necrosis as seen microscopically. Until additional evidence has been accumulated it is proposed that neoplasms having five or more mitotic figures per ten high-power fields (HPF) be tentatively termed epithelioid leiomyosarcoma or leiomyosarcoma with epithelioid features and those with less than five mitotic figures per ten HPF, epithelioid leiomyoma.

Adult

Gastric epithelioid leiomyoma and leiomyosarcoma (leiomyoblastoma).

A series of 127 surgical specimens of epithelioid leiomyomatous tumors (leiomyoblastomas) of the gastric wall from the files of the Armed Forces Institute of Pathology (AFIP) were studied as to biologic behavior, morphogenesis, and histologic features of value in distinguishing benign and malignant variants. These tumors affect middle-aged men primarily and usually present with upper gastrointestinal bleeding or peptic ulcer-like symptoms. They are composed of a mixture of round epithelioid and spindle cells, many of which have clear cytoplasm. The cells are ensheathed by delicate reticular fibers. The presence of a perithelial or glomoid pattern in some tumors suggests a possible relationship to angiomyoma, glomus tumors, and "pericytoma." The epithelioid leiomyoma, the benign form, often arises in the mid- and distal stomach, especially on the anterior wall. Microscopically, it is recognized by the presence of large epithelioid cells and infrequent mitotic figures. Of 103 epithelioid leiomyomas, only one metastasized and thus was biologically malignant. The epithelioid leiomyosarcoma often arises in the proximal stomach and also distally, especially on the posterior wall. Two histologic types of epithelioid leiomyosarcoma are distinguished from the benign epithelioid leiomyoma by the small size of the cells and occasional higher mitotic counts. One sarcoma variant is a small cell caricature of the leiomyoma. The other is more anaplastic, assoicated with a loss of reticular fibers surrounding the cells and an alveolar arrangement. Epithelioid leiomyosarcomas are the most common type of gastric sarcoma. They are aggressive neoplasms; 63% metastasized, usually within 2 years after diagnosis.

Adolescent

Estrogen and progesterone binding proteins in normal human myometrium and leiomyoma tissue.

The occurrence and characteristics of macromolecular components of normal human myometrium and leiomyoma which bind [3H]estradiol and [3H]progesterone were investigated, employing dextran coated charcoal, density gradient centrifugation and gel filtration techniques. On sucrose density gradient centrigugation, [3H]progesterone was bound by macromolecules with sedimentation rates of about 4 S and 8 S. The major [3H]progesterone binding component had a sedimentation coefficient of about 4 S, which contained specific and nonspecific binding sites. Sedimentation patterns as well as elution profiles from agarose gel revealed a striking similarity between biochemical properties of the progesterone receptors from normal myometrium and leiomyomas of the same organ. Both progesterone and estradiol receptor change in concentration during the normal menstrual cycle. During the early proliferative phase the number of estradiol receptor binding sites was highest; after ovulation, a rapip decrease of estradiol receptor level was seen. On the other hand, using [3H]progesterone as the ligand, the highest receptor concentration was found at midcycle. The leiomyoma steroid hormone receptor levels were compared with those in normal myometrium. Whereas leiomyoma exhibited higher estradiol binding capacity, the concentration of progesterone receptors was low in fibroid tumors.

Carrier Proteins

Uterine leiomyoma: correlation between signal intensity on magnetic resonance imaging and pathologic characteristics.

To correlate the signal intensity of uterine leiomyoma with its pathologic characteristics, with particular emphasis on the fibrous component, 33 magnetic resonance (MR) examinations that revealed 93 leiomyomas were prospectively studied. All patients were imaged in axial and sagittal planes with different spin-echo pulse sequences to obtain T1-, T2-weighted, and proton density images. Nondegenerative leiomyomas (n = 62) showing a homogeneous signal of low intensity, and degenerative leiomyomas (n = 31) with a heterogeneous signal of variable intensity on T2-weighted images could be correlated. Histopathological assessment of fiber constitution and degeneration, and MR intensity were interpreted by independent observers. There was excellent accord between the averages for MR intensity, T2 relaxation time and fiber content, although the intensity values in each fiber grade showed a wide range. The greater the fiber content the lower the MR intensity on T2-weighted images, and the shorter the T2 relaxation time (p < 0.0001). In addition, the manner in which fiber distribution affected MR appearance was also elucidated. These data contribute guidelines for precise tissue differentiation of myogenic tumors on MR images, and for MR imaging tissue diagnosis of any lesion with a considerable fibrous element.

Adult

Leiomyomas of the larynx.

In a now 65 years old male patient a monochorditis of the left vocal cord was clinically observed over several years. Multiple exploratory excisions finally demonstrated a leiomyoma with atypically growing histological structures. The complaints of the patient permanently increased, and a hazel-nut-sized leiomyoma with atypically growing tissue structures, originating in the left vocal cord, was removed by laryngectomy. The exploratory specimen, which was diagnosed in our laboratory as proliferative pseudosarcomatous tissue 11 months before manifestation of the leiomyoma with atypical structures, is compared with analogous findings reported by Lane (1957) and Sherwin et al. (1963). Our observations, completing the results of these publications, allow the conclusion that pseudosarcomatous tissue structures do not only occur in the surroundings of carcinomas but also in the immediate neighbourhood of mesenchymal tumours. Choice of the adequate site for exploratory excision is decisive for correct histological diagnosis. A comparison with the literature concerned shows that leiomyomas and leiomyosarcomas rarely occur in the laryngeal region.

Aged

Vascular leiomyosarcoma: the malignant counterpart of vascular leiomyoma.

The clinical and pathological findings of six cases of leiomyosarcoma arising from blood vessels of different caliber are described. The term vascular leiomyosarcoma, having both a topographic and morphologic significance, is proposed for these tumors. The histologic pattern is characterized by a proliferation of atypical smooth muscle cells with a large number of intermingled blood vessels. Mitoses were counted per 10 high power field (hpf) and tumors were divided in three groups I, 10 to 20 mitoses, group II, 20 to 35 mitoses, and group III, more than 35 mitoses per 10 hpf. The mitotic index seems to be the most important pathological feature on which a prognostic evaluation for vascular leiomyosarcoma can be based. Tumors in group I had neither local recurrences nor metastases; the one tumor in group II had one local recurrence, but the patient is free of disease 6 years after surgical treatment; the three tumors in group III developed distant metastases and constitutional symptoms. Vascular leiomyoma, bizarre leiomyoma, and hemangiopericytoma are included in the differential diagnosis of vascular leimyosarcoma. The possibility that vascular leiomyosarcoma arising from small vessels represents the malignant counterpart of vascular leiomyoma is proposed.

Adult

Benign metastasizing uterine leiomyoma. Multiple lymph nodal metastases.

A case of histologically benign lymph nodal metastases from a uterine leiomyoma in a 27-year-old woman is reported. It is postulated that fragments of a leiomyoma, detached at the time of endometrial curettage, entered dilated lymphatic channels in or adjacent to a large projecting submucous leiomyoma, and seeded several pelvic and para-aortic lymph nodes. During an interval of 8 years, these grew slowly and did not infiltrate the perinodal tissues or give rise to secondary metastases.

Adult

Leiomyomas of the rectum and anal canal: report of six cases and review of the literature.

Since 1872, 168 rectal leiomyomas have been reported in the world literature. Their incidence, therefore, is very low. It is estimated that one leiomyoma may be seen for every 2,000 or more rectal tumors. Those leiomyomas located in the anal canal and sphincter are the rarest. A case is reported here that appears to be the eleventh in such a location. The incidence of these lesions is highest between the ages of 40 and 59 years. Most patients have a combination of symptoms rather than a single one, the most frequent of these being the presence of a mass, bleeding, and constipation. Almost always, the chain of events that leads to the diagnosis starts when the tumor is discovered by digital examination of the rectum. The lack of reliable criteria of malignancy and the marked tendency to recur shown by these tumors are the two main problems the surgeon faces when selecting the operative procedure to be used. We believe that those tumors with an original size of 5 cm or more in largest diameter are the ones that have shown the highest tendency to recur, mostly as sarcomas. Therefore it is thought that these lesions should be treated radically from the beginning, especially when they recur. More adequate follow-up studies are needed.

Adult

delta 4-3-Ketosteroid 5 alpha-oxidoreductase in human uterine leiomyoma.

Homogenates of human endometrial, myometrial, and leiomyoma tissues were incubated with (1,2,6,7-3H)-testosterone, and 3H-dihydrotestosterone was subsequently identified, indicating the presence of the enzyme delta 4-3-Ketosteroid 5 alpha-oxidoreductase. The enzyme activity of leiomyoma was higher than that of endometrial and myometrial tissue, while endometrial activity was higher than myometrial activity. The results raise the possibility that 5 alpha-reduced androgens may play a role in the pathophysiology of uterine leiomyoma.

3-Oxo-5-alpha-Steroid 4-Dehydrogenase

Leiomyoma of the female urethra.

Leiomyoma of the urethra is a rare condition, which is more common in female than in male subjects. There are only 14 documented cases of leiomyoma of the female urethra in the literature. A careful analysis of these cases revealed the average patient age to be 34.1 years. The site of the lesion determined the clinical features and the surgical approach. No common etiological factor could be ascertained. Two additional cases of leiomyoma of the female urethra are reported.

Adult