Chordomas are locally aggressive or malignant tumors that involve the slope and sacrococcygeal region. Although the growth of chordoma is slow and distant metastasis is rare (metastasis can occur in late stages), it is still a malignant tumor because it is very destructive locally, because it continues to grow and is very likely to recur after surgery.
Epidemiology
Chordoma is rare, accounting for 1% to 4% of primary malignant bone tumors, and is more common in men. It was named by Ribber in 1894 and has an overall incidence of 0.2 to 0.5 per 100,000 per year, accounting for about 0.15% of intracranial tumors. The disease occurs most often in infants.
Etiology
Chordoma is a congenital tumor that develops from embryonic residual chordal tissue.
The notochord is the embryonic mesodermal tissue located in the central dorsal region, which later grows into the skull base and spinal column, and the residual notochord tissue is the source of chordoma. Chordoma occurs in the midline at both ends of the spine. It presents with osteolytic distending destruction. In the early stages, the tumor surface is lobulated or nodular, with a dry tumor size, incomplete envelope, and gray or grayish red color. The tumor tissue may have residual fragmented bone or small septum interval, soft tissue calcification, easy hemorrhage, necrosis and cystic degeneration in the late stage, with single lesions being common.
Chordoma has a tendency to spread inside and outside the dura mater, to the subarachnoid space and to the perineural area, and can cause uncontrollable pain. Rarely, if ever, does it metastasize until years after the tumor is discovered. Metastases are generally seen only in sacrococcygeal chordoma. Metastases are more likely to occur in those who have received radiation therapy.
Clinical manifestations of chordoma
Pathogenesis
Chordoma presents as a smooth nodule with white translucent jelly-like tumor tissue containing large amounts of mucus that is dark red when accompanied by extensive bleeding. The edges of the tumor are often lobulated or nodular, and the surface is covered with a fibrous tissue envelope that generally does not penetrate into the adjacent organs. Microscopically, the tumor cells are small, square, round or polygonal, with clear cell membranes and red-stained vacuoles, which can be dozens of times larger than the normal cell volume. The nucleus is round or ovoid and centrally located. The cells are arranged in cords or irregular glandular cavities and are mucus-like with occasional large deep-stained nuclei, multinucleated cells and nuclear schwannoma cells.
Chordoma can be divided into two types, classic and chondrogenic sacral invasion, which can invade the pelvis forward and the spinal canal backward, compressing the cauda equina nerve root and causing symptoms of nerve root damage in the corresponding area.
Clinical manifestations
Chordoma is most commonly seen in middle-aged and elderly people aged 40-60 years old and occasionally in children and young adults. The tumors are found at both ends of the spine, i.e. skull base and sacral vertebrae, 35% in the former and 50% in the latter, and 15% in other vertebrae. Rarely, they occur outside the longitudinal bones, such as transverse vertebrae and sinus bones. More often, sacrococcygeal pain is the first symptom. The vast majority of intravertebral chordomas tend to experience associated symptoms for months to years prior to diagnosis. Clinical symptoms are determined by the location of the tumor: tumors in the occipital pterygoid region may produce headaches with cerebral nerve compression (optic nerve is the most common), destruction of the pituitary gland may have pituitary dysfunction protruding laterally or inferiorly may form a mass in the nasopharynx with obstruction of the nasal cavity with purulent discharge. If the tumor occurs in the lower slope and craniocervical junction, headache, pain in the occipital or occipitocervical junction area are common symptoms, and the symptoms can be aggravated when the head position is changed; if the tumor occurs in the thoracic spine, it can invade the vertebral structure of the corresponding area and protrude into the thoracic cavity through the intervertebral foramen and destroy the intercostal nerve, which can cause segmental burning neuralgia and even pulmonary pleural irritation. If the tumor occurs in the sacrococcygeal region, the symptoms of sacral tumor compression appear later and often the main symptom is pain in the sacrococcygeal region. The typical symptom is chronic back and leg pain, with persistent nighttime aggravation. If the mass occurs in other parts of the spinal canal, localized pain in the corresponding area is a common symptom.
Diagnosis of chordoma
Sacral chordoma is clinically seen as a full sacral area on examination, and the tumor can be palpated on anal examination as round and smooth with some elasticity. Most slow-growing tumor masses swell and grow anteriorly and are not easily detected clinically only in late stages when the tumor breaks backward into the gluteal muscle, sacrospinous muscle or subcutaneously, and the mass can also be palpated in the lower abdomen. Anal finger diagnosis is a routine examination for early detection of sacral tumor, especially for patients with chronic lower abdominal pain that has not been cured for a long time and is suspected to have sacral tumor.
In infancy, soft compressible masses occur along the midline of the face, skull or back, which may be transilluminated or enlarged by crying. The cutaneous manifestations of spinal atresia include depressed damage dermal damage abnormal pigmentation damage hair damage polypoid damage tumor, subcutaneous tissue and vascular damage.
Chordoma presenting with metastases include lung, eyelid and penis 87 cases of surgically treated sacral chordoma, 5 cases presenting with pulmonary metastases along with 1 case of pelvic lymph node metastasis and 1 case of tibial chordoma.
Complications: incontinence of urine and feces may be combined.
Clinical manifestations
Headache is the most common symptom. The nature of headache is persistent dull pain, often a full headache, or it may extend to the occipital or cervical region.
Chordoma of the saddle: hypopituitarism, mainly manifested as impotence, amenorrhea, body fat, etc.; optic nerve compression produces primary optic nerve atrophy, vision loss and bilateral temporal hemianopia, etc.
Parasympathetic chordoma: The main manifestations are kyphosis, talipes, and adductor nerve palsy, with adductor involvement being more common.
Chordoma of the slope: The main symptoms are brainstem compression, i.e., walking impairment, cone bundle sign, abduction, and facial nerve impairment. As the tumor occurs at the base of the skull, it may cause traffic hydrocephalus, for example, the tumor develops toward the pontocerebellar horn, hearing impairment, tinnitus, vertigo, and if it originates distal to the nasopharyngeal wall, it often protrudes to the nasopharynx causing nasal non-ventilation and pain, and purulent or bloody secretions are seen.
Diagnosis
The diagnosis can generally be made based on the characteristic clinical manifestations of lesion characteristics and imaging examinations. The tumor is located in the sacrococcygeal spine and produces various compression symptoms.
Giant cell tumor of bone, neurofibroma and chordoma are all common tumors occurring in the sacrum. They have the same clinical symptoms, and the same osteolytic destruction on X-ray is easily confused with each other and needs to be differentiated, but the first two are mostly in young adults aged 20 to 40 years old. Other rare benign tumors are easily identified by their own imaging features on radiographs due to mild symptoms. The more rare sacral high malignant tumor has a short history, severe pain affecting the sleep position, the patient soon develops mental depression, weight loss, emaciation, anemia and fever.
Auxiliary examination
1.X-ray examination
X-ray plain film shows that the tumor is mainly osteolytic destruction without calcification and ossification. Local destruction of sacral bone and its calcified plaque can be seen. Tumors located in sacral and caudal vertebrae produce limited bone destruction from the central or partial side of sacral vertebrae, which may cause bone expansion and thinning.
2.Cystogram and barium enema
It is helpful to judge the scope of tumor.
3.CT examination
CT is of localization and qualitative value to determine the tumor, and it is of great value to find calcification or plaque formation in the tumor, and it can guide the surgery after intravenous injection, and it can help to clarify the contents of the tumor and its surrounding envelope characteristics. CT can clearly show the relationship of chordoma bone destruction and soft tissue shadows to the cauda equina, large blood vessels, and surrounding tissues. According to the literature, cystic changes of chordoma can be shown as speckled and hypodense areas in CT; angiography can be helpful in the diagnosis of cervical chordoma; myelography can show the epidural extension of tumor in the spinal canal, and the growth of tumor in the spinal canal can be beyond the bone destruction, which is helpful in the development of surgical plan.
4. MRI scan, magnetic resonance examination has localization and qualitative value for tumor, and is a very useful tool for evaluating chordoma. MRI should be performed routinely when bony destruction is found on CT scan. Chordoma has a low signal on T1 image or isosignal on T2 image with a high signal lobulated high signal lesion clearly separated from the low signal. It is important to note that MRI can differentiate tumor type generally classical chordoma shows longer T1 and T2 signals than chondrogenic chordoma.
Treatment
With the advances and improvements in surgical techniques, successful surgical resection of sacral chordoma is now possible and most patients are cured. Radical surgical resection plays a major role in the treatment of chordoma, and the site of the tumor determines the surgical approach. No single surgical approach is appropriate for all patients with chordoma. Chordoma of the craniocervical junction can be obtained through a lateral anterior or posterior approach to obtain appropriate resection of sacral chordoma. If the tumor is invasive to sacral 1, total sacral resection is feasible, and the large pelvic vessels must be carefully protected during artificial pelvic replacement, and intraoperative hemorrhage causing hemorrhagic shock must be prevented according to Enneking surgical staging. The tumor is excised with less contamination and the sacral nerve is freed from the tumor and the bone shell above the tumor is scratched, which significantly reduces recurrence after surgery.
Chordoma treatment
Preservation of sacral
Preservation of the sacral 12 nerve is associated with urinary and fecal incontinence in 50% of patients (Enneking) The author’s experience is that preservation of the sacral 123 nerve results in normal continence and bilateral lower extremity function in more than 90% of patients. Loss of sacral 4 and 5 nerves can cause temporary sensory disturbances in the perineum and some male patients have temporary sexual dysfunction. Urinary and fecal dysfunction that occurs after surgery with sacral 12 and 3 nerve damage on one side recovers after 2 to 4 months.
Sacral tumors are extremely blood-rich surgical resection because of large surgical exposure difficult bleeding and high risk of complications high morbidity and mortality rate was often regarded as a forbidden area in the past. In continuous exploration, it was recognized that total sacral tumor resection is a salvage operation that must be fully prepared before it can be performed.
In the past, when sacral tumors could not be removed surgically, radiotherapy had the function of pain relief and tumor development control. Small doses of postoperative radiotherapy are useful to kill the remaining tumor cells.
It is difficult to cure chordoma with surgical treatment alone. Because a tumor of bone origin usually precludes total resection, the rate of tumor recurrence remains high even after radical resection of the tumor. The main reason may be the progressive growth of the residual microscopic tumor.
Postoperative radiotherapy often has a different outcome. However, chordoma is not sensitive to radiotherapy, so the ideal dose of postoperative radiotherapy has been the subject of clinical sensitivity. investigators have concluded that there is no correlation between high-dose radiotherapy and length of survival. Although the literature reports differently, doses of at least 5000 rad are generally chosen when conventional external radiation therapy is used.
Early CT or MRI examination after chordoma resection to confirm the extent of tumor resection and the presence of tumor remnants is an important guide to the decision to use postoperative adjuvant radiotherapy or regular follow-up.
Prognosis
The overall prognosis for chordoma is poor, but the prognosis for chondrogenic chordoma is generally considered to be better; Heffelfinger and colleagues reported that the average survival for chondrogenic chordoma was four times that of patients with classic chordoma. In their data, only one patient with classic chordoma survived longer than 10 years, whereas approximately 50% of patients with chondrodysplasia survived longer than 10 years. Surgical resection is not complete and is prone to recurrence, but distant metastases are less common.
Recipe
Recipe: 1. 9g of Radix Codonopsis Pilosulae, 9g of Radix Astragali, 9g of Radix Astragali, 9g of Radix Paeoniae Alba, 9g of Rhizoma Atractylodes Macrocephalae, 12.5g of Rhizoma Atractylodes, 31g of Radix Astragali, 9g of Radix Bupleuri, 31g of Radix Oyster, 12.5g of Radix Xia Gu Cao, 6g of Pericarpium Citri Reticulatae, 5g of Radix Mucuna Pruriens, 12.5g of each of Seaweed and Kelp (decoction). At the same time, Erhuangwan (five-percent package) was added and one capsule was swallowed weekly.
Effect: One case of osteolytic sarcoma was reported to be cured.
2. Internal formula: Xuan Hu, Boswellia, Myrrh, Salvia, Safflower, Liu Yao Nu, Niubizi, Radix et Rhizoma, Yi Mu Cao, 9g each, 6g each of Sumac and Blood Dried, and 3g of Turmeric, decocted in water. Externally: 12.5g of Angelica Sinensis, 9g of Radix et Rhizoma Paeoniae, Rhizoma Cyperus, Rhizoma Polygonatum, Rhizoma Liu, Rhizoma Blood, 6g of Boswellia, 6g of Myrrh, 2g of Saffron, 3g of ice chips, 0.15g of Musk. powdered and applied to the affected area for 3 days, and then reapplied with new medicine.
Effect: One case of giant cell tumor of bone was reported to be cured.
Prescription: 30g of coix seeds, 30g of green beans, 30g of adzuki beans, cooked like porridge, eat the beans and drink the soup.