MESOTHELIAL TURIORS the Mesoderm of the Embryo Separates
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MESOTHELIAL TURIORS CHARLES F. GESCHTCKTER, M.D. (From the Siirgicd Piithologictil LaDorat ory, Deportmelit of Surjityv, Johns fZopkins Elo~pitaland Univerhity) The mesoderm of the embryo separates early into two major divisions. A paraxial or somatic portion forms the sclerotome. A coelomic or visceral por- tion forms the splanchnocoele and the tissue for the genito-urinary organs. Both the somatic and the visceral mesoderm give rise to epithelial and mesen- chymal elements. In the somatic mesoderm the primitive myo-epithelium is replaced by voluntary muscle; the mesenchyme forms the various types of con- nective tissue, including cartilage, bone, fibrous tissue, and fat. In the coelomic or visceral mesoderm the epithelial derivatives include the meso- thelium in the serous cavities and the epithelium of the genito-urinary organs; the mesenchymal elements form smooth muscle and angioblastic tissue from which are derived the vessels, lymphoid and myeloid elements. The major divisions and derivatives of the mesoderm are indicated in the outline below: SOMATICMESODERM Myo-epithelium ......................... Voluntary muscle Mesenchyme ............................ .Connective tissues (cartilage, bone, etc.) VISCERALMESODERM Mesenchyme ........................... Angioblastic tissue, smooth muscle Coelomic epithelium .................Mesothelium, genito-urinary epithelium The splanchnocoele, the major derivative of the coelomic cavity, gives rise to the special serous cavities of the body, including the peritoneal, pericardial and pleural cavities, which are lined by persisting coelomic epithelium-the mesothelium. Persisting portions of mesodermal partitions which once di- vided the coelomic chamber form the mesentery. A tendency for the coelomic epithelium to persist as such and for the underlying mesoderm to form vascu- lar connective tissue rather than muscle is characteristic of the derivatives of the splanchnocoele. The tumors derived from the pleura, pericardium, and peritoneum, to which the name mesothelioma is given, show similar tendencies. Their components are epithelial and fibrous in nature. The more malignant tumors are extremely vascular (Fig. 1). In the head region of the embryo, the mesoderm, after the appearance of the cerebrospinal fluid, forms the meninges. These structures, like the lin- ings of the serosal cavities, are composed of connective tissue overlaid by a single layer of epithelium. While attempts have been made to relate the meninges to mesenchyme or to neurectoderm rather than to mesothelium, the histologic structure of these membranes is similar to that of the serosal linings, showing a combination of epithelial and fibroblastic components. Like meso- 378 FrG 1. PHOTOMICROOKAPI1 Or MESOTLILLIOMA 01 1JiL PLEURA SllOWI\G THE CIIARACTERISTICS OF VAXULARlTY, EPITIILLIAL PROLll1 RATTOY AYD SPINULL-CEI L S4RCOAIA. PATH. NO. 38568 FIG.2. PHOTOMICROGRAPHOF A VASCULAR MENINGEAL TUMOR OCCURRII~G IN A MANOF SIXTYYEARS The tumor perforated both tables of the sltull. Path. No. 53069. 379 3 80 CHARLES F. GESCHICKTER thelium the large flat cells on the outer surface of the pia and on the arach- noidal surfaces may be outlined by silver nitrate. The more undifferentiated neoplasms of the meninges are extremely vascular (Fig. 2). The largest number of mesothelial tumors occur in the meninges; they are benign in character and are variously termed arachnoidal fibroblastomas, dural endotheliomas, and meningiomas. The pleural tumors are next in frequency but are more malignant in character. Tumors of the pericardium and peri- toneum are extremely rare and are similar in behavior to those of the pleura. In the formation of the ovary the primitive mesoderm is carried inwardly with the down-growing epithelium and from it tumors of a mesothelial character are occasionally formed. Metastases are extremely rare among the mesothelial tumors, regardless of location. The following tabulation presents the inci- dence of these tumors in the Surgical Pathological Laboratory of the Johns Hopkins Hospital. Meningeal tumors .......................................... 106 Pleural tumors ......................................... 25 Pericardial and peritoneal tumors ............................. 3 Ovarian mesotheliomas .................................... 2 TOTAL 136 MENINGEALMESOTHELIOMAS Meningeal tumors comprise about one-sixth of the new growths involving the cranial contents. Similar tumors may arise from the coverings of the spinal cord. The varied terminology applied to these growths reflects the dif- ferences of opinion regarding the origin of the leptomeninges from the meso- derm or neurectoderm and the relation of these neoplasms to the arachnoid or dura. The majority of meningeal tumors are encapsulated growths occurring in adult life. In the present series of 106 cases the peak of age incidence was in the decade between thirty and forty. Sixty-five per cent of the patients were between thirty and fifty years old. The youngest patient was fourteen, the oldest sixty-two. The tumors grow slowly and may remain symptomless for many years. The average duration of symptoms in the present series was two years and a quarter, although a history extending beyond five years was not rare. The growths are most common near the vertex along the longitudinal sinus, in the falx, about the temporal lobe, or at the sphenoidal attachments about the sella turcica. Intracranial pressure (evidenced by headache, vomiting, and impaired vision) and epileptic seizures, usually beginning in the leg or arm, are the most common signs. The intracranial pressure may increase very slowly and the headaches may be relatively mild in character. Tumors near the longitudinal sinus produce convulsions, usually beginning in the arm or leg, and motor aphasia. Changes in personality may occur. Lesions in the falx are characterized by weakness in the leg or clonic seizures in this extremity. The roof of the corresponding ventricle appears depressed in the ventriculogram. Neoplasms arising near the clinoid processes of the MESOTHELIAL TUMORS 381 sphenoid or the lesser wing produce blindness and may be characterized by primary atrophy of the optic nerve on the affected side and choked disc on the opposite side. The lesions in the olfactory groove produce anosmia and uncinate attacks. The symptoms associated with spinal meningeal tumors vary with the level of the cord compressed. Paraplegia and bladder dis- turbances are outstanding. The locations of the tumors in the present series were as follows: Frontal region .................................... 30 Region of falx ..................................... 18 Temporal and middle fossa .............................. 15 Spinal meninges ........ ......................... 10 Pontine angle .............. ..................... 7 Parietal region ............. ..................... 4 Occipital region ................................... 4 Suprasellar region ..... ......... .......... 4 Sphenoidal region ........ ....... ............ 4 Multiple ..................................... An interesting feature of these meningeal tumors is the variety of changes produced in the cranial bones. In the roentgenogram may be seen markings produced by increased vascularity, clearly demarcated areas of bone resorp- tion, and centers of calcification. In about 10 per cent of the cases cranial osteophytes or hyperostoses are produced by invasion of the bones; the tumor passes through both inner and outer tables and produces radiating spicules of new bone from both. Such cranial involvement must be differentiated from benign osteomas, which arise during childhood, produce mound-like swelling over the outer table and depress but do not invade the inner table, In the present series there were 10 cases of hyperostoses produced by meningeal tumors. These and 18 similar cases gathered from the literature have been analyzed by Echlin. In some instances the new bone production was not discovered until operation. In these cases the average duration of symptoms at the time of operation was five years or about twice as long as the average for the entire series. The bones involved, in the order of frequency, were frontal, parietal, frontoparietal and temporal. In 4 cases thickening of orbital plates produced exophthalmos. In approximately one-half of the cases the bone involvement rather than the intracranial growth was the first finding noticed by the patient. Except for the visible and palpable swelling, the symptoms produced do not differ from those of meningeal tumor without bone involvement. While the typical roentgen picture shows spicules of bone radi- ating from both tables, these may be absent in the very early stages, and the bones show only a rounded thickening. At operation the meningeal tumor is found attached to the dura and ex- tending inwardly, pressing upon the brain substance. In the majority of cases the tumor does not extend to the tables of the skull or excite new bone forma- tion in the region overlying the tumor. The tumor itself, however, may be gritty on section, enclosing calcified or osseous areas. The overlying dura and cranium show increased vascularity. The following case is illustrative of the more invasive meningeal tumors producing osteophytes and erosion in the cranial bones: FIG.3. ROENTGENOGRAMOF AN INVASIVEMENINGEAL TUMOR PENETRATING THE SKULLAND PRODUCINGOSTEOPIIYTES IN TIIE CRANIALBONILS. PATH. No. 45782 FIG.4. CZSE SIIOWN IN F~G.3: A VIEW' OF INTERIOROF SKULL;B. SPECIMENSlIOWING RELATIOK OF TIIE TUMORTO THE FALXAND