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Education TUMORS of MUSCLE CHARLES I? Education TUMORS OF MUSCLE CHARLES I?. QESCHICKTER, M.D. (From the Surgioal Pathological Laboratory, Department 07 Surgery, Johns Hopbilcs Hoapital and Uniuereity) INTRODU~TION: EMBRYOLOGY The muscular structures of the body appear very early in the em- bryo and are derived from mesoderm. Within the first month of embi:yonic life the mesoderm spreads out from the hind end of the embryo and separates into a paraxial mass, an intermediate cell mass, and sheets applied to the body wall and primitive digestive tube (Fig. 1). In the paraxial mesoderm and in the primitive body wall the seg- WCURAL CANAL ORGANS c.-u. ARCYENTCRON JCLlNCWNO- PLCURC FIG. 1. nIAGRAMMATIC SECTION OF VERTmRATE EMBRYOSHOWING THE PARTS OF THE MESO- DERM (Modifled from Koith’s Human Embryology, Wm. Wood 5, Go., 1933) ment a1 voluntary muscles are developed. In the intermediate cell mass the musculature connected with the urogenital organs develops. In association with the splanchnopleure applied to the primitive gut, the muscles of the digestive tube, the heart, and the large blood vessels develop. Tumors are extremely rare in the spinal muscles of the body wall and limbs derived from the paraxial mesoderm. This rarity of tumor formation is probably dependent upon the extremely early differentia- tion of these structures. According to Keith no more new fibers are formed in skeletal muscle after birth, muscular growth henceforth being effected by enlargement of pre-existing fibers. On the other hand, tumors are very common in the smooth muscle of the genito- urinary organs developed from the intermediate cell mass, Here cle- 378 TUMORS OF MUSCLE 379 velopmental processes are delayed and modified in association with the sex physiology which necessitates fairly rapid changes in these structures. In the musculature of the digestive tube, heart, and large blood vessels derived from the splanchnopleure tumor incidence occu- pies a mid-position. New growths here are neither so rare as in the vol- untary muscles nor so common as in the musculature of the gcnito- urinary organs. TABLMI: Tumora of Smooth Muscle Urogenital Digestive Vascular Tumor Musculature Musculature Musculature Leiomyoma Uterus.. ... 5,900 G. I. tract., . , .128* Leiomyoma cutie Vagina. ...220* 2 Intestines. .... 45* 4 with involved Bladder.. .. 48* 1 Rectum ....... 27, 1 vessels. ......... 7* Prostate.. 7* 7 Stomach.. .... 19 Kidney.. .. 4 Esophagus .... 28* Leiomyosarcoma Uterus.. ... 54 Digestive tract 20, Heart. ........... .40* Bladder.. .. 19* 6 Stomach.. .... 1 Ileum. ....... 1 Rectum. ...... 1 - Rhabdomyoma and Uterus.. ... 18* 1 Heart.. .......... .42* Rhabdomyosarcoma Cervix.. ... 1 Vagina. ... 2 Bladder.. .. 13" 1 Testicle.. .. 14* Myoblastoma Vagina. , . 1 * Collected from the literature. Other numbers represent cases on file in t,he Johns Hopkins Hospital. The individual muscle cells develop from a syncytial stage in the mesenchyme. These cells form fibers of smooth muscle, according to Carey, by the drawing out of their spongioplasm into longitudinal processes. Where voluntary muscle is formed there is a definite myo- blastic stage in which the elongated cells become multinucleated and protoplasmic processes develop, in which striae appear. Between the third and fourth months of embryonic life the centrally arranged nuclei of these myoblasts migrate to the surface and both the nucleus and cytoplasm are encased in a sarcolemma sheath to form an adult fiber of voluntary muscle. According to Carey, the distinction between smooth and voluntary muscle is a matter of degree of differentiation in response to the laws of dynamics. Smooth muscle is formed from mesenchyme by a tension exerted on the muscle cells during growth. If this tension is much increased, smooth muscle is converted into voluntary muscle, Carey was able to convert smooth muscle of the bladder in the young dog into striated muscle by increasing the tension on the bladder wall with fluid pressure. Tumors of smooth muscle repeat the histogenesis of this structure. 380 CHARLES F. OESCEIOHTER Myosarcoma and Rhabdomyosarcoma M yoblastoma Non-indigenous tumors Myoarcoma with giant Oral cavity. ........... .12*-1 Myoaitk ossificans .........32 mlls .................12 Tongue. ...............22*-l Lipoma. .................. 1 Rhabdomyosarcoma. .... 2 Subcutaneous. .......... 7*-1 Fibroma.. ................10 Extremities.. ........... 2*-5 Angioma.. ................ 3 Sacral (subcutaneous). ... 3 Mammary .............. 2 "Collected from the literature. Other numbers represent eases on file in the Johns Hopkins Hoepital. Tumors of striated muscle and cardiac muscle repeat the more com- plicated histogenesis of these tissues. Benign tumors of striated muscle show cross striations in their elongated cytoplasmic processes (rhabdo- myoma). More undifferentiated benign tumors composed of myoblasts with wavy, granular fibrils also occur. The sarcomas of striated muscle in certain cases may duplicate in structure the spindle-cell sarcomas of smooth muscle or may show the features of the multinucleated myo- blastic stage in which the cells are large, elongated, and multinucleated. The histologic types of muscle tumors and their distribution in the muscular structures are indicated in Tables I and 11. I. TUMORSOF INVOLUNTARY~IUSCLE Leiomyoma Uterine Myomas: These muscular tumors, the most frequent of uterine neoplasms, occur during the period of active sex life. Approxi- mately 6,000 of these tumors have been surgically removed at the Johns Hopkins Hospital in the last forty years, They are more common in the colored than in the white race. The tumors are either single or multiple and may be (1) subserous, projecting from the wall of the uterus.toward its peritoneal surface, (2) intramural, within the uterine wall, or (3) submucous, directed toward the uterine cavity. The subserous and submucous varieties may be pedunculated and cause acute symptoms by twisting and strangulation of the pedicle. Necrosis, infection, and fibrosis are common changes in these growths. Myoma may caum increased menstrual bleeding through changes produced in the neigh- boring endometrium, or the submucous type of myoma may bleed into the uterine cavity through erosion of the vessels. Small myomas may produce no symptoms. Diagnosis can usually be made by palpation. The mass can be moved backward and forward with the uterus and has TUMORS OF MUSCLE 38 1 a characteristic nodularity or firmness. Under the microscope strands of smooth muscle and adult connective tissue predominate. These may be displaced by cysts, calcareous areas, or hemorrhage. Rarely marked proliferation occurs, and in about one per cent of these tumors malignant change in the form of spindle-cell sarcoma (leiomyosarcoma) is seen. (Figs. 2-6.) FIG.2. BENIQNMYOMA OF THE UTERUS,SHOWING THE ENCAPSULATEDNATURE OF TEE GROWTH AND THE FIRM,INTERTWININQ BANDSOF MUSCLEAND FIBROUSTISSUE. PATH. NO. 37884 FIQ. 3. PHOTOMI('R0QRAPH OF A BENIQNMYOMA OF THE UTERUS, SHOWINQ INTERLACINO FIBERSOF SMOOTH MUSCLE. PATH.No. 44834 Myomas are treated by irradiation, myomectomy, or hysterectomy. Radiation is preferred in intramural growths of moderate size in women near the menopause, but is contraindicatcd in younger women in the childbearing period since the regression obtained is brought about by the effect of the radiation on the ovaries. In these younger patients, and where the uterine mass is larger than a three months' pregnancy, 382 OHARLES F. OESCHICKTER myomectomy should be performed. Necrotic and infected tumors pro- ducing marked symptoms in older women or undergoing malignant change are treated by hysterectomy. In all cases where conservative measures are contemplated diagnostic curettage is indicated to exclude malignancy. Myomas of Other Gewito-Urinary Organs: Myomas in the genito- urinary organs other than the uterus are not uncommon. Myomas of the broad ligament and of the cervix are usually associated with similar lesions of the uterus. Schilling was able to collect 220 cases of myomas of the vagina. The tumors are usually found in the anterior vaginal wall in patients between thirty and forty-five years of age. The larger krowths are pedunculated; they may press on the bladder or rectum FIG. 4. RAPIDLY PEOLIFERATING MYOMAOF TEE Umus IN A WOMAN OF TWENTY-FOUB, REMOVEDBY HYSTE~ECTOMYIN 1928. PATH.No. 41514 The patient was reported well in July 1934, six yeare later. and are treated by excision. Kretschmer collected 48 leiomyomas of the bladder and found them equally divided between males and females. Hematuria, frequency, and dysuria were the common symptoms. The lesions can apparently appear at any age. From his study Kretschmer estimated that 10 per cent of all bladder tumors were mesothelial or embryonic in origin. Hinman and Sullivan collected six cases of leio- myoma of the prostate. The lesions occur in adults and are usually mistaken for prostatic hypertrophy (Fig. 7). In approximately 13,000 autopsies performed at the Johns Hopkins Hospital there are recorded one leiomyoma of the bladder, 4 in the kidney, and 6 in the prostate. In the cases surgically treated one my- oma of the prostate, two of the vagina, and one attached to the ovary were observed. The majority of the lesions were small and definitely encapsulated. (Fig. 8.) Myomas of the Digestive Tube: Benign tumors of smooth muscle may occur in the wall of the digestive tract anywhere in its course from the esophagus to the rectum. Bouvier in 1924 collected a series of 128 leiomyomas occurring in the digestive tube. They are more common
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