Myxomatous Tissue Research Articles

Odontogenic myxoma: ultrastructural and histochemical studies.

An odontogenic myxoma of a maxilla has been examined. Histochemistry of the mucosubstance indicated that hyaluronic acid and chondroitin sulphate were present. On ultrastructural examination many of the cells in the myxomatous tissue were seen with prominent rough endoplasmic reticulum, suggesting a secretory function, and possibly the myxomatous ground substance was produced by these cells. Cells containing collagen fibrils were found. Epithelial islands with intercellular spaces producing an arrangement similar to the stellate reticulum of the enamel organ were found, and possibly had developed from odontogenic epithelial rests. These islands were surrounded by a clear zone, outside which were cells with an increased prominence of adenosine-triphosphatase, nucleoside-diphosphatase, thiamine-pyrophosphatase, and often arylamidase reaction products. This probably represents a reaction of the mesodermal tissue to the epithelial islands. Possibly the epithelium exerts an inductive effect on the mesodermal tissue, and the myxomatous appearance may be the result of an aberrant development of mesodermal cells into ;myxoblasts' which secrete the myxomatous ground substance.

Clinical and pathological findings in patients with "floppy" valves treated surgically.

Fifty-one patients underwent operation for "floppy" aortic or mitral valves during a three-year period between 1969 and 1972. Aortic valve disease occurred predominantly in men, while mitral valve lesions were equally distributed between men and women. In all groups, the mean age at operation was in the fifth decade; only patients with Marfan's syndrome came to operation before the age of 30 years. The clinical course was characterized by rapid deterioration in left ventricular failure after the onset of symptoms; an asymptomatic murmur was often present for many years prior to decompensation. Hemodynamic findings showed a wide range of abnormalities, with cardiac index consistently reduced to 1.5 L/m 2 /min ± 0.3 in aortic disease, and 1.6 L/m 2 /min ± 0.4 in mitral disease. At operation the aortic cusps were thin and prolapsing and, in 5 of 11 patients, an aneurysm of the ascending aorta required resection. Mitral valves were soft, voluminous, with elongated and ruptured chordae. Prolapse of the cusps or billowing of a flail segment produced mitral regurgitation. In 3 patients, mitral valve replacement was complicated by acute dissection of the aorta at the site of the aortic clamp; this was diagnosed at operation and successfully repaired in 2 and discovered at autopsy in 1. Histology of the valves confirmed the presence of diffuse myxomatous tissue in all cusps, cystic medial necrosis in the aorta, and varying degrees of collagen disorganization and necrosis in chordae tendineae. There was frequently deposition of similar myxomatous tissue around chordae and on the ventricular endocardium, possibly as a response to friction. Forty-four patients survived operation and have been followed up for periods up to 36 months. There were 2 late deaths, and 2 valves were replaced at a second operation. There has been no evidence of valve dehiscence. It is concluded that heart disease resulting from connective tissue abnormalities is unique in its clinical course, the high incidence of associated aortic aneurysms and ruptured chordae tendineae, and the postoperative complications of valve dehiscence. Homograft replacement has given encouraging results.

Embolism From Cardiac Myxoma

To the Editor.— I wish to point out the fallibility of a priority claim inThe Journal, in the article entitled Ventricular Myxoma Associated With Cerebral Embolism ( 214 : 2154, 1970)—specifically, the claim in the summary to the effect that an embolus associated with a myxoma of the ventricle has not been previously reported. Articles by Danta and Williams, 1 Young and Hunter, 2 and Kay et al 3 would indicate otherwise. The first mentioned cites other instances of embolism due to ventricular myxoma; the third reports surgical removal of a myxoma in a patient with recurrent embolism proved to be due to myxomatous tissue.

Congenital pulmonary stenosis resulting from dysplasia of valve.

A distinctive type of pulmonary valvular stenosis, termed "pulmonary valvular dysplasia," is described from six necropsies and 10 living children. The anatomic features of the stenotic pulmonary valve are unique in that there are three distinct cusps and no commissural fusion. The obstructive mechanism is related to markedly thickened, immobile cusps, characterized by the presence of disorganized myxomatous tissue. Several clinical features tend to distinguish this form of pulmonary stenosis. In each of 16 patients studied, a pulmonary ejection murmur was present, but this was not associated with an ejection click. Other features suggestive of this type of pulmonary stenosis were slow body growth, abnormal facies, and a positive family history of pulmonary stenosis. The electrocardiogram showed a greater degree of right axis deviation than is found in most cases of dome-shaped pulmonary stenosis. Right ventriculography revealed distinctive features of the pulmonary valve which were characterized by lack of typical dome-shaped deformity and by the presence of thick cusps. Experience with simple incision-valvulotomy was associated with a high (38%) operative mortality and significant residual stenosis in survivors. The suggested operative procedures include excision of a valve cusp, placement of a right ventricular outflow patch, or replacement of the pulmonary valve.

Chemical and histochemical characterization of mucopolysaccharides in a jaw myxoma.

Chemical and histochemical analyses, including testicular and staphylococcal hyaluronidase digestion, have been made of a jaw myxoma and the results show the presence of two acid mucopolysaccharides. Of the total mucopolysaccharide present 80% was hyaluronic acid and 20% chondroitin sulphate. The high content of non-sulphated mucopolysaccharide would seem to explain the paucity of fibres characteristic of the myxoma. It is suggested that myxomas generally probably have a similar high hyaluronic acid content. It is considered that the cell concerned is a mesenchymal cell elaborating non-sulphated mucopolysaccharide and may be called a ;myxoblast'; it is metabolically different from the sulphated-mucopolysaccharide-collagen-producing fibroblast. The high hyaluronic acid content is much greater than that found in embryonic connective tissue and may be a significant factor in the neoplastic behaviour of the myxomatous tissue. The aggressive behaviour of the myxoma is against a simple reversion to embryonic mesenchyme. It is concluded that the myxoblast is an active mucopolysaccharide-secreting cell and that mucin in the myxoma is not a sign of cell degeneration of preexisting fibroblasts or collagen.

Benign mesenchymoma of the heart in the guinea pig. A report of four cases.

Thirty-six hundred ninety-eight guinea pigs of the Dunkin-Hartley strain from the breeding colony at Fort Detrick were necropsied. Four females had mesenchymomas of the heart. The tumors were similar histologically and were composed of multiple benign mesenchymal tissues which were amalgamated into a single mass. One tumor was composed of fibrous, angiomatous, adipose, cartilagenous, osscous, hematopoietic, myxomatous, and possibly smooth muscle tissues. Smooth muscle and myxomatous tissues were not found in the other tumors. All were benign and probably originated from the primitive mesenchyme of the heart.

Myxosarcoma of the skin.

AMYXOMA consists of stellate or spindle-shaped cells in a loose matrix which contains mucoid material (most likely hyaluronic acid), reticulum, and collagen fibers. Thus, histologically, myxoma resembles primitive mesenchymal tissue and umbilical cord matrix. Myxomatous tissue may occur in the skin in several benign conditions. Ganglion is a term describing a cystic tumor-like lesion microscopically similar to myxoma, found near joints and tendons in the subcutaneous tissues. Papular and localized plaque-like accumulations of myxomatous nature are found in the skin in hypothyroid and hyperthyroid states and also where no thyroid pathology is demonstrable, as in lichen myxedematosus (papular mucinosis). 1 In addition, various neoplasms of the skin (sarcoma, fibroma, lipoma, etc.) may show appreciable amounts of myxomatous tissue. Malignant myxoma, or myxosarcoma, is similar microscopically to the benign types described above, but the stellate cells show variations in shape, in size, and in staining of

Hamartoma of the lungs

I N 1904 Albrecht' described instances of biliarv harnartomas He defined as tumor-like malformations in which occur only abnormal mixing of the normal components of the organ The abnormality may take the form of change in quantity, arrangement, or degree of differentiation, or may comprise all three. The deduction to be drawn from the histologic examination of these formations is that they have originated in abnormal mixing of their development . The lesions have been variously reported as pa.pillomas, fibroadenomas, chondromas, mixed tumors, hamartoma chondromatosum, adenochondrouras and lipochondromas. Many of these tumors embrace all of the bronchial anlagen, both mesodermal and endodermal tissues Albrecht designated all of these mixed tumors as hamartomas. So-called cliondromas of the lung are in reality hamartomas and, according to Jones,° they constitute 0.25 per cent of all routine autopsy cases McDonald, Harrington and Clagett' report twenty hamartomas in a series of 7,972 necropsies, an incidence of 0.25 per cent These chondromas of the lung contain bronchial epithelium in clefts between cartilaginous masses and may also include the mucoid, fibrous, lymphoid and muscular tissues The term hamartoma. should be reserved for this type of lesion Hickey and Simpson' reviewed forty cases of primary chondroma of the lung and added two cases of their own Microscopic examinations of their own cases indicated a composition of islands of cartilage, myxomatous connective tissue, adipose tissue, mature fibrous connective tissue and papilliferous gland-like structures, covered by a single layer of non-ciliated cuboid or columnar epithelial cells In some areas the tumors were covered by stratified ciliated epithelium and contained lime salt deposits Generally the tumors contained the structures previously enumerated, although in a few instances osseous tissues were present

RETROPERITONEAL MYXOLIPOMA

A case of a fatty tumor of the retroperitoneal space, with the histologic diagnosis of myxolipoma, is presented. The boy was 7 years old and weighed 24.2 kg.; the tumor weighed 12.8 kg., or more than half the child's weight. The clinical history suggests that the tumor appeared at the age of 1½ years and developed without any complications of the renal and intestinal functions. There was predominance of the myxomatous tissue and the removal of the whole tumor was possible. It was located between the mesenteric leaves and for its removal, 50 cm. of the ileum had to be resected. The complete extirpation was performed and the child is in good physical condition six months after the operation.

Uterine mixed tumor of nine years' duration

1. 1. A case of uterine mixed tumor with an unusually long time of survival is presented. 2. 2. The surgical specimens removed at the onset of the disease and nine years later were both characterized by epithelial elements as well as by heterologous stromal tissue. Our histologie study suggests that in uterine mixed tumors not only the stromal but also the epithelial elements are derived from embryonal myxomatous tissue. 3. 3. The clinical and histologie features of our case support Wilms' cell rest theory of mixed tumors.

Treatment of Malignant Tumors of the Testis

The incidence of malignant testicular tumors is relatively low. It has been estimated by Cade that they constitute 1 to 1.5 per cent of all malignant tumors in males and that they make up about 3 per cent of neoplasms of the genito-urinary tract. Pack and LeFevre reported an incidence of 1 per cent among 16,565 patients with malignant tumors seen at Memorial Hospital, New York, while Dean at the same institute in 1935 found 2.09 per cent of all malignant neoplasms in males to be testicular tumors. In regard to all male hospital admissions, it may be mentioned that Tanner in 1922 found one testicular tumor for every 2,000 patients. The present study is based on the tumors of the testis treated at the University of Minnesota Hospitals from 1926 to 1943 inclusive. The total number amounted to 100 and they were placed in three groups: 34 cases were not classified as to type, while 37 were seminomas and 29 were carcinomatous mixed tumors. Etiology The average age in this series was for seminoma 37.6 years and for carcinomatous mixed tumors 29.4 years. This is in agreement with the statement of Cade that teratomatous tumors occur chiefly between twenty and forty years, and the seminomas between thirty and fifty. A history of trauma was obtained in 15 per cent of the cases. This is somewhat lower than in series reported by other authors. Russell Howard obtained a history of trauma in 5 cases out of 27, Nash and Leddy in 21 out of 103. The figures of some authors are as high as 50 per cent. Cryptorchidism was present in 8 cases. Nash and Leddy reported an incidence of 8.7 per cent and Dean found 35 cases in a series of 245, or 14.3 per cent. Hydrocele was observed in 6 patients, and in 3 of these a malignant tumor of the testis was discovered during the operation for hydrocele. Classification Classification of malignant tumors of the testis is a confused subject, and opinions vary greatly. At the University of Minnesota Hospitals we follow E. T. Bell's classification. Bell states: “Testicular tumors may be divided into four groups: adult teratomas, carcinomatous mixed tumors, seminomas, and chorionepitheliomas. “1. Adult teratomas may be typical dermoid cysts containing hair, fatty material, teeth, etc., or they may consist of many small cysts separated by solid tissue. These tumors are rare, develop slowly over many years, and offer relatively good prognosis. “2. Carcinomatous mixed tumors are soft cellular growths consisting chiefly of epithelium in form of cysts or solid masses of carcinomatous structure. Cartilage and myxomatous tissue may be found scattered through the tumor. “3. Seminoma is the most common form of testicular neoplasm. It consists chiefly of rounded or polyhedral clear cells arranged in thick solid cords and bearing some resemblance to testicular tubules. Little or no cartilage is present. “4. Chorionepithelioma is a malignant tumor arising from chorionic epithelium.

MUCOUS GLAND TUMORS OF THE SKIN

Mucous gland lesions of the skin are rather uncommon in general dermatologic experience. We believe that more of them would be recognized if histologic preparations were made oftener of bizarre cutaneous lesions which are usually destroyed therapeutically without being studied. Alertness as to their embryonic origin should lead to more frequent clinical suspicion of their presence. In a review of the literature of so-called mixed mucous and salivary gland tumors in the skin and the subcutis, Ahlbom1pointed out that the most frequent site is the face but that a few are found on the trunk and the extremities. He treated three such tumors at Radiumhemmet, in Stockholm, Sweden, two on the face and one on the sole of the foot, and observed a certain degree of malignancy in all of them histologically. He expressed the belief that those tumors which consist partly of myxomatous and cartilage-like tissue are

Fibrous osteoma of the jaws

Thirteen cases of fibrous osteomas of the jaws are reported and the pathology of two other recorded cases discussed. The bone was formed by the process of fibrous or membranous ossification, cartilage being seen in a minute trace in only one case. In twelve cases the tumor consisted largely of cancellous bone and fibrous marrow. In three it consisted largely of fibrous tissue in varying degrees of maturation, and ossification was proceeding slowly. There was also myxomatous tissue present in three cases and occasional small islands rich in giant cells in two. Microscopic signs of inflammation were rarely present.

FIBROUS OSTEOMA OF THE JAWS

Summary Thirteen cases of fibrous osteomas of the jaws are reported and the pathology of two other recorded cases discussed. The bone was formed by the process of fibrous or membranous ossification, cartilage being seen in a minute trace in only one case. In twelve cases the tumor consisted largely of cancellous bone and fibrous marrow. In three it consisted largely of fibrous tissue in varying degrees of maturation, and ossification was proceeding slowly. There was also myxomatous tissue present in three cases and occasional small islands rich in giant cells in two. Microscopic signs of inflammation were rarely present.

Myxosarcomata: With a Report on 51 Cases Studied at the State Institute for the Study of Malignant Disease, Buffalo, New York

A perusal of modern texts on pathological anatomy gives us but little information on the subject of the myxosarcoma. A few sentences, or at most a short paragraph, usually suffice for description of this type of neoplasm, and the statements made are often misleading or more than usually vague. Yet during the past century a voluminous literature has been published on, and a vast amount of research devoted to, the myxomatous tumors. Early to attract the attention of pioneers in pathological anatomy, these neoplasms have, through succeeding generations, presented for solution problems which still remain unsolved. The fact that myxomatous or pseudomyxomatous tissue at times appears in many different types of tumor further complicates the study. Maximow makes the definite statement that mucous connective tissue is not present in adult mammals, and continues: “It is found during the development of the embryo in many places of its body, as under the skin, and is a form of the common, loose, irregular connective tissue.” Early investigators made intensive studies of this type of tissue in the gelatinous matrix of the umbilical cord, Wharton's jelly. Frey and others thought that they could demonstrate that the cord is composed of a cellular reservoir with anastomosing tubes on which could condense, in surrounding them, a system of channels, resulting from the condensation of the mucous substance. Thus each cell or branch would occupy the axis of a young connective tissue fiber which would completely enclose it. The interior of these channels would be filled with mucus, containing here and there some embryonic cells, destined later to form fat cells. This conception was valiantly contested by Renaut, who brought evidence to show that the mucin of the cord is not confined in canals or lymphatics, but that it is found in cords or strands of varying size, on which the connective-tissue cells are placed as plaques. He also showed that these connective-tissue cells, fibroblastic in type, did not form a complete sac for the mucin or jelly. It is quite probable that the apparent canalization of Wharton's jelly, and the formation of strands in it, may have been due to the preparation of tissue for microscopic study and did not represent actual conditions found during life. “The intercellular substance [of the cord] is soft, jelly-like, and homogeneous in fresh condition; when fixed, it contains granules and fibrillar precipitates. It gives the reaction for mucin” (Maximow p. 94).

THE MASTOID CELLS: THEIR ARRANGEMENT IN RELATION TO THE SIGMOID PORTION OF THE TRANSVERSE SINUS

The placing of the mastoid operation by Schwartze on a rational anatomic basis awakened a tremendous interest in the study of the development of the mastoid. The first studies consisted chiefly of descriptions of the structure of the mastoid. The next advance in the knowledge of this structure came with an inquiry into the development of the air spaces. The fundamental basis for the present knowledge of the pneumatization of the mastoid must be credited to Wittmaack. His work has been augmented by the more recent researches of Albrecht and Schwarz. I shall not go into the detailed description of the successive stages of development of the tympanum, attic, antrum and mastoid cells. Stated simply, according to Wittmaack's¹theory, the pneumatization of the temporal bone is the result of a transformation due to the successive displacement of the myxomatous embryonic tissue in the preformed cavities by the epithelium. The

A Papillary Mixed Tumor of the Body of the Uterus

Mixed tumors of the uterus vary markedly in structure, and for that reason some confusion has arisen in the terms used in discussing these growths. Theoretically any tumor of the uterus containing at least one mesoblastic tissue foreign to those of the uterus belongs in this group. On the basis of such reasoning, lipomas, osteochondromas, and composite growths of carcinoma and sarcoma as reported by Jarfe (6), Schiffmann (15), and others, would be included. There seems to be no concise definition of the group. Perlstein (10), Ritter (13), and Gamper (3) discuss these growths as mixed mesodermal or mesenchymal tumors of the uterus. They include the occasional tumors with epithelium but exclude those that are benign. Halter (5) spoke of heterotopic mixed tumors, irrespective of embryonal origin or structural characteristics, while Jones (7) and Cox and Benischek (1) chose the descriptive term, “sarcoma botryoides.” Others designate the tumors as rhabdomyosarcoma and chondromyxosarcoma. In none of the reports has much consideration been given to the mono-, bi-or tridermal composition of these bizarre tumors—factors concerned with origin—or has the dearth of epithelial derivatives been especially emphasized. There is such a wide variety of mixed tumors, each individually different, that it is possible here to indicate only their more important features. Tumors of the uterus commonly referred to as “mixed” are of more complex structure and usually contain sarcoma-like tissues. Petersen (11) collected thirty fatty tumors of the uterus, but these generally are not considered complex enough to be included in the mixed group; they are seldom malignant and only occasionally contain striated muscle, cartilage, or embryonic myxomatous tissue. Other simple mesoblastic tissues, as the angiomas, and the more complex teratomas are excluded, as are the atypical growths of the cervix described by Geller (4), which contain only modified epithelium.

FIBROMYXOMA—PROBABLY A LINEAR NEVUS

The following case is reported as one that is apparently unique in medical annals. A careful search of the literature on nevus at the Library of the Army Medical Museum did not reveal a case in which myxomatous tissue had been found. The literature on myxoma and fibromyxoma is scant. No clue to a tumor of similar distribution can be found in textbooks or in current literature. CASE REPORT History.— E. D., a colored woman, aged 33, married; a laundress, gave an unsatisfactory family history. Her past history was negative. She had had no previous skin disease and no other serious illness. She had had no children. Her present illness began at birth. Examination.— The condition was located on the left arm, beginning about 3 inches below the axilla and extending to the center of the palm. On the under surface of the arm, as it was extended with the