Arch Orthop Trauma Surg (2000) 120 : 544–547
© Springer-Verlag 2000
CURRENT PROBLEM CASE
A. A. Kurth · J. Warzecha · M. Rittmeister · E. Schmitt · L. Hovy
Recurrent chondroblastoma of the upper thoracic spine A case report and review of the literature
Received: 23 April 1999
Abstract A very rare case of recurrent chondroblastoma of the upper thoracic spine with a follow-up of more than 20 years is presented and discussed with a review of the literature. Key words Spinal tumors · Chondroblastoma · Recurrence
Introduction Chondroblastoma is a benign, cartilaginous tumor that usually arises in the epiphysis of long bones during the 2nd and 3rd decades. The most common sites are the proximal tibia, proximal and distal femur, and proximal humerus. Chondroblastoma of the spine is extremely rare, and only 12 cases have been reported in the literature.
Case report In 1977, a currently 62-year-old man complained of left scapula pain radiating into the left arm down to the 4th and 5th fingers. The complaints were misdiagnosed as musculoskeletal dysfunction of the shoulder girdle, and the initial treatment was conservative. Physical therapy did not improve the symptoms. During a routine checkup of the lung, a tumor of the upper thoracic spine at T8 was found, which had infiltrated the upper lobe of the lung. A thoracotomy was performed in June 1978, and the intraoperative macroscopic and histological findings on the spine were interpreted as an aneurysmal bone cyst. The subsequent histological examination of the spinal tissue diagnosed a giant cell tumor. As a complication of surgery, the patient developed a Horner syndrome on the left side and a quadrant syndrome with anhydrosis of his left
A. A. Kurth () · J. Warzecha · M. Rittmeister · E. Schmitt · L. Hovy Department of Orthopedic Surgery, University Hospital Frankfurt, Section for Spinal Disorders and Spinal Surgery, Section for Orthopaedic Oncology, Marieneburgstrasse 2, D-60528 Frankfurt/Main, Germany e-mail:
[email protected], Tel.: +49-69-6705228, Fax: +49-69-6705375
shoulder. Following surgery, the patient continued to suffer from pain in his left arm. A myelography performed in December 1979 showed a slightly narrowed spinal canal at the level of the first and second thoracic vertebrae without compression of the spinal cord. Concomitant angiography revealed a dense net of tumor vessels at that level, and scintigraphy defined the extent of the tumor process to be localized to T1 with a maximum activity in the left transverse process. A biopsy of that lesion including a hemilaminectomy of T1 was performed. Histological results of the material obtained showed the typical morphological features of a benign chondroblastoma. A total resection of the tumor was planned, but preoperative computed tomography (CT) revealed the tumor mass infiltrating the vertebral body and arch of T1, the left upper lung and the left paraspinal muscles of the cervicothoracic junction. In October 1980, a resection of the tumor mass of T1 and a posterior fusion from C7 to T2 with internal fixateur and bone grafting were performed. The tissue retrieved was macroscopically soft and dark stained and was histologically classified as a benign chondroblastoma. In May 1981, a new CT exam revealed a large recurrence of tumor mass extending from C7 to T2, reaching the spinal canal. Progression of spinal canal compromise made it necessary to resect the tumor again. Histology revealed a benign chondroblastoma. During all this time the patient never ceased to suffer from chronic pain requiring special pain management. In May 1987, a routine exam revealed new destruction of C7 necessitating fusion from C6 to T3 to stabilize the spine, to remove the recurrence which affected the vertebral body of T1 completely and partially affected T2 and C7. Histological examination again showed the chondroblastoma. The next recurrence of the tumor at T1 and T2 was revealed by magnetic resonance imaging (MRI) in February 1992. The spinal tumor was removed via a transthoracic approach in March 1992, and again a benign chondroblastoma was histologically confirmed. Furthermore, in July 1992 the posterior elements of the tumor recurrence were removed through a dorsal approach to the spine. When the patient was last seen by us in 1996, he complained of increasing pain in his neck and left arm. MRI revealed a tumor mass of T1 with affection of C7 and T2 as well as tumor manifestation in the spinosus processes of C6 and T1. Suspicious-appearing infradiaphragmal structures were interpreted as possible lymphomas. The resection of an 8 × 2 cm spinal tumor mass was performed from a dorsal approach. Plans were made to remove a mediastinal tumor mass in a second operation. Retrieved tumor tissue was also sent to a pathologist specialized in bone tumors for a second opinion. He confirmed the diagnosis of chondroblastoma and also described a malignant transformation in some parts of the specimen. Between the onset of pain and the patient’s last hospital stay for recurrence of the spinal tumor, more than 20 years had passed.
545
Discussion Chondroblastoma at spinal and extraspinal sites The chondroblastoma is a well-known tumor first described by Ewing as a calcifying giant cell tumor in 1928 [14] and shown by Codman to be of cartilaginous origin [5]. The chondroblastoma accounts for about 1% of primary bone tumors [6, 21, 26]. It is considered to be benign with a good prognosis in the majority of the cases, but in some cases a metastatic spread is reported [16, 25, 27]. Three apparent types of chondroblastomas are described based on their behavior of growth. The first seldom relapses following curettage and bone grafting. The second, more uncommon type has identical histological features but behaves like a malignant tumor with rapid and destructive growth and a tendency to multiple relapses and metastasis [27, 41]. The third and least common type shows malignant transformation [1, 6, 18, 32, 37, 40]. As the occurrence of a malignant metastasizing chondroblastoma is extremely rare, it is not mandatory to remove each chondroblastoma radically [27]. About 90% of the patients is cured by initial curettage or local excision of the tumor followed by filling the defect with bone grafting [6, 27, 30]. After adequate therapy, the local relapse rate is on average between 5% and 10% [1, 6, 30, 34, 39]. Higher rates of relapse have been reported especially if the tumor has an aneurysmal bone cyst component [22, 23, 24]. Chondroblastomas usually appear in the epiphysis or epi-metaphysis or in the apophysis of long bones like the femur, tibia or humerus [1, 6, 9]. Radiological features include epiphyseal location (98%), open epiphysis (70%), an osteolytic lesion with or without punctate calcifications (25%–60%); sclerotic borders are seen in 60% [9]. An erosive buckling and increased slenderness of the corticalis is seen more often in short marrow and flat bones than in long bones [1, 6, 15, 26, 28, 29, 35]. Destruction of cortical bone and invasion of the surrounding soft tissue as presented in our patient is considered to be atypical and mostly associated with uncommon tumor localization and extensively progressed tumors [26, 30]. Macroscopically, the tissue of the chondroblastoma is elastic-robust and colored gray-blue. Histologically, it shows differently sized, knotty formations of chondroid matrix, which contain sharply separated chondroblasts with isomorphic nuclei [33]. Chondroblastomas of the spine The chondroblastoma of the spine is a very rare benign tumor [12]. To our knowledge only 12 cases have been reported in the literature; 6 reported cases of chondroblastoma in the cervical spine exist: In 3 of them, a chondroblastoma occurred simultaneously at two different levels, once at C1 and C2 [43], once at C3 and C4 [11], and once at C5 and C6 [19]. In the 4th case, it occurred in C5 [30] and in the 5th and 6th cases [2, 10] it was localized at C7. Chondroblastomas of the thoracic spine were found at
Fig. 1 Localisation of the 12 cases of a spinal chondroblastoma reported in the literature
T3/4 [3] and T6/7 [15]. Three cases of chondroblastoma in the lumbar spine [1, 4, 26] and 1 occurrence of chondroblastoma in the sacrum [38] have been reported. No prevalence in the age of patients suffering from a chondroblastoma was found in literature. Most studies report its presence in younger men in their second decade [1, 4, 6, 24, 26, 30, 34]. Ages are reported in 9 of 12 cases of spinal chondroblastoma. Seven patients were male and aged 9 [2], 12 [11], 16 [19], 17 [43], 28 [3], 29 [38] and 59 [15] years. The two remaining patients were female and aged 16 [30] and 24 [10] years. The male patient presented here was 44 years old when he first displayed symptoms. The average age when spinal chondroblastoma first occurs is 25.2 years. Clinical findings suggestive of this tumor are somewhat nonspecific, commonly being pain of the affected vertebral bodies, especially to palpation [10, 19], pain in the interscapular and scapular region [3, our case], and a feeling of tightness of the neck muscle [19]. Neurological symptoms, like pain radiation into the fingers as found in our patient, are described by others as well [3, 10, 19]. The time period from the occurrence of first symptoms to the diagnosis of a spinal chondroblastoma range from a few months to several years [3, 6, 22, 26, 30, 34]. In the case presented, a chondroblastoma was diagnosed not earlier than 2 years following the onset of symptoms. The differential diagnosis of a spinal chondroblastoma include osteoblastoma, aneurysmal bone cyst, chordoma, eosinophilic granuloma, giant cell tumor, clear cell chondrosarcoma, spondylitis, chondromyxoid fibroma and metastasis of an extraosseous primary tumor. The osteoblastoma, which can show features of an aneurysmal bone cyst [4, 7, 30] and the true aneurysmal bone cyst most commonly appear in the vertebral arch and affect the vertebral body secondarily [4, 30, 34]. The chordoma affects both vertebral body and arch [4, 15, 29, 30]. An early and typical radiological sign of chordoma is destruction of the disc and associated narrowing of the intervertebral space [8, 15,
546
29]. Chordomas of the cervical spine often start out at the second vertebra [8] and proceed to adjacent vertebrae [30]. The eosinophilic granuloma develops in vertebral bodies as well, showing up on X-ray as uniform and rapid flattening of the vertebral body [4, 15, 30, 34]. The giant cell tumor of the spine has an osteolytic behavior and commonly proceeds from the body to the arch [4, 6, 30, 36]. The spine is an extremely rare location for an osteosarcoma [4, 6, 24, 30, 34]; it has a predisposition for the vertebral body and seldom spreads into the arch [4, 13, 30]. Spondylitis and other vertebral infectious diseases may show features on CT and MRI similar to our presented case of spinal chondroblastoma. Spinal chondroblastomas have been incorrectly diagnosed as atypical tuberculosis [3], eosinophilic granuloma [11], aneurysmal bone cyst [43], chondrosarcoma [15], chordoma [19], and osteoblastoma [30]. In our case, histological examination of the specimen obtained from each surgery repeatedly revealed the welldefined chondroblastoma. The last histology from August 1996 showed tissue with numerous cells and chondroid matrix, as seen in 95% in a series of 495 chondroblastomas from the Mayo Clinic [26]. The cells in our case were uniform and of medium size with poor eosinophilic cytoplasm and with enlarged polymorphic and grooved nuclei, as was seen in 11% of the Mayo Clinic series of chondroblastomas. Among those cells, we found groups of giant cells with several nuclei and focal degenerative transformations as well as groups of siderophere macrophages as described by Kurt et al. in 24% of his reported tumor cases [26]. Scattered small blood vessels as in our case are seen in only 1.4% of tumors. We did not find calcified deposits as seen in 35% of the Mayo Clinic series. One of our specimens had spindle cell proliferating components with scattered mitoses, which was interpreted as suspicious of malignant transformation. Mitotic figures are seen in 77% of all chondroblastomas. Cortical and soft-tissue penetration as in our case is seen in 5%. It is not surprising that histology of the first removed specimen in June 1978 showed only the characteristics of an aneurysmal bone cyst. This must be regarded as a typical sign of a chondroblastoma, because in 20% of cases a dominant aneurysmal bone cyst component may mask the features of chondroblastomas. An aneurysmal bone cyst component is found in 38% of all chondroblastomas. Histologically, a chondroblastoma must be differentiated from giant-cell tumors because osteoclast-like cells usually form a part of the pattern of a chondroblastoma, being present particularly at the border of the chondroid areas of the tumor. The presence of mononuclear cells with multinucleated giant cells is responsible for the misinterpretation as a giant-cell tumor of bone [4, 6, 17, 37]. In fact, for a long time, it was designated as the calcifying epiphyseal variant of a giant cell tumor [14]. As with chondroblastomas, giant-cell tumors of the spine are extremely rare, but if they affect the spine, it usually occurs in a skeletally immature patient. [17, 30]. The clinical course of our patient’s disease was characterized by non-invasive growth of a histologically con-
firmed chondroblastoma and by multiple relapses. Finally, the chondroblastoma seemed to transform into a malignant tumor. The clinical course of chondroblastomas to the spine has been reported in five cases. Ehalt and Ratzenhofer [11] described a 12-year-old boy with a chondroblastoma in the cervical spine; that tumor progressed after surgery. Despite the tumor showing evidence of progression, the patient was asymptomatic for 15 years after the operation. Buraczewski et al. [3] related a case of chondroblastoma of T3 and T4 in a 28-year-old man showing no evidence of disease 2 years after laminectomy. Brasse et al. [2] reported one case of a 9-year-old boy who suffered a relapse at level C7 1 year after laminectomy; he underwent radiation therapy and has remained free of any relapse ever since. In another case, a 20-year-old man suffered three relapses after tumor resection [38], and Edel et al. [10] reported a case of a 24-year-old woman with no evidence of recurrence 7 months after curettage. Considering those cases and the clinical course of our patient, it must be assumed that there is a more frequent rate of relapse in spinal chondroblastomas than in chondroblastomas at extraspinal sites. Although there has been no case of metastasizing spinal chondroblastoma, they seem to grow more aggressively and invasively than those at extraspinal sites; they behave similarly to tumors of type II or III. At least partially, chondroblastomas of the spine fulfill the criteria of malignancy and should initially be treated more radically compared with chondroblastomas at extraspinal sites.
References 1. Bloem JL Mulder JD (1985) Chondroblastoma: a clinical and radiological study of 104 cases. Skeletal Radiol 14: 141–149 2. Brasse F, Schmitt M, Hoeffel JC, Montant J, Bey P, Bretagne MC (1985) A propos d’une observation de chondroblastome vertebrale. Rev Chir Orthop 71: 1043–1047 3. Buraczewski J, Lysakowska J, Rudowski W (1957) Chondroblastoma (Codman’s tumor) of the thoracic spine. J Bone Joint Surg Br 39: 705–710 4. Campanacci M (1990) Bone and soft tissue tumors. Springer, Berlin Heidelberg New York 5. Codman EA (1931) Epiphyseal chondromatous giant cell tumors of the humerus. Surg Gynecol Obstet 52: 543–548 6. Dahlin DC, Ivins JC (1972) Benign chondroblastoma: a study of 125 cases. Cancer 30: 401–413 7. Dahlin DC, Unni KK (1986) Bone tumors: general aspects and data of 8542 cases, 4th edn. Charles C. Thomas, Springfield, Ill., USA 8. Davidson JK, Mucci B (1985) Case report 322. Skeletal Radiol 14: 76–80 9. Earl W, Brien JM, Mirra RK (1997) Benign and malignant cartilage tumors of bone and joint: their anatomic and theoretical basis with an emphasis on radiology, pathology and clinical biology. Skeletal Radiol 26: 325–353 10. Edel G, Ueda Y, Roessner A, Blasius S, Müller-Miny H, Erlemann R (1992) Chondroblastome des Kopfes und der Wirbelsäule. Osteologie 2: 81–94 11. Ehalt W, Ratzenhofer M (1967) Zur Kasuistik des gutartigen Chondroblastoms. Z Orthop 102: 625–629 12. Enderle A, Schmitt E, Zichner L (1981) Diagnosis of spinal diseases – a critical review. Z Orthop 119: 193–205 13. Erlemann R, Reiser M, Roessner A, Peters PE, Grundmann E (1987) Primäre Knochentumoren und tumorähnliche Läsionen der Wirbelsäule. Eine Analyse radiologischer Dignitätskriterien. RöFo 147: 131–137
547 14. Ewing J (1928) The classification and treatment of bone sarcoma. Report of the International Conference on Cancer (Bristol). J Write, pp 265–267 15. Freyschmidt J, Ostertag H (1988) Knochentumore. Klinik, Radiologie, Pathologie. Springer, Berlin Heidelberg New York 16. Green P, Whittaker RP (1975) Benign chondroblastoma. Case report with pulmonary metastasis. J Bone Joint Surg Am 57: 418–419 17. Greenspan A, Remagen W (1998) Differential diagnosis of tumor and tumor-like lesions of bone and joints. Lippincott Raven, Philadelphia 18. Hatcher CH, Campbell JC (1951) Benign chondroblastoma of bone. Its histologic variations and a report of late sarcoma in the site of one. Bull Hosp JT Dis 12: 411–430 19. Howe JW, Baumgard S, Yochum TR, Sladich MA (1988) Case report 449. Skeletal Radiol 17: 52–55 20. Hudson TM, Hawkins JF (1981) Radiological evaluation of chondroblastoma. Radiology 139: 1–10 21. Huvos AG (1979) Bone tumors. Diagnosis, treatment and prognosis. Saunders, Philadelphia 22. Huvos AG, Marcove RC (1973) Chondroblastoma of bone. A critical review. Clin Orthop 95: 300–312 23. Huvos AG, Marcove RC, Erlandson RA, Mike V (1972) Chondroblastoma of bone: a clinicopathologic and electron microscopic study. Cancer 29: 760–771 24. Huvos AG, Higinbotham NL, Marcove RC, O’Leary P (1977) Aggressive chondroblastoma. Clin Orthop Rel Res 126:266–272 25. Khalili K, White LM, Kandel RA, Wunder JS (1997) Chondroblastoma with multiple distant soft tissue metastases. Skeletal Radiol 26: 493–496 26. Kurt AM, Unni KK, Sim FH, McLeod RA (1989) Chondroblastoma of bone. Hum Pathol 20: 965–976 27. Kyriakos M, Land VJ, Penning HL, Parker SG (1985) Metastatic chondroblastoma: report of a fatal case with a review of the literature on atypical, aggressive and malignant chondroblastoma. Cancer 55: 1770–1789 28. McLeod RA, Beabout JW(1973) The roentgenographic features of chondroblastoma. Am J Radiol 118: 464–471 29. Meyer JE, Lepke RA, Lindfors KK, Pagani JJ, Hirschey JC, Hayman LA, Momose KJ, McGinnis B (1984) Chordoma: their CT appearance in the cervical, thoracic and lumbar spine. Radiology 153: 693–696
30. Mirra JM, Picci P, Gold RH (1989) Bone tumors, clinical, radiologic and pathologic correlations, Vols 1 and 2. Lea Febiger, Philadelphia 31. Monda L, Wick MR (1985) S-100 protein immunostaining in the differential diagnosis of chondroblastoma. Hum Pathol 16: 287–293 32. Reyes CV, Kathuria S (1979) Recurrent and aggressive chondroblastoma of the pelvis with late malignant neoplastic changes. Am J Surg Pathol 3: 449–455 33. Riede UN, Schaefer HE, Wehner H (1989) Allgemeine und spezielle Pathologie. Thieme, Stuttgart 34. Schajowicz F (1981) Tumors and tumor-like lesions of bone and joints. Springer, Berlin Heidelberg New York 35. Schulze K von, Treugut H, Müller GE (1980) Benignes Chondroblastom-malignes radiologisches Bild. RöFo 132: 450–454 36. Schwimer SE, Basset LW, Mancuso AA, Mirra AM, Dawson EG (1981) Giant cell tumor of the cervico-thoracic spine. Am J Radiol 136: 63–67 37. Sirsat MV, Doctor VM (1970) Benign chondroblastoma of bone. Report of a case of malignant transformation. J Bone Joint Surg Br 52: 741–745 38. Spjut HJ, Dorfmann HD, Fechner RE, Ackermann L (1971) Tumors of bone and cartilage. Atlas of tumor pathology, fascicle 5. Armed Forces Institute of Pathology, Washington DC 39. Springfield DS, Capanna R, Gherlinzoni F, Picci P, Campanacci M (1985) Chondroblastoma: a review of seventy cases. J Bone Joint Surg Am 67: 748–755 40. Steiner GC (1966) Postradiation sarcoma of bone. Cancer 18: 603–612 41. Van Horn JR, Vincent JG, Wiersma-Van Tilburg AM, Pruszczynski M, Slooff TJJH, Molkenboer JFWM (1990) Late pulmonary metastases from chondroblastoma of the distal femur. A case report. Acta Orthop Scand 61: 466–468 42. Vestring T, Edel G, Müller-Miny H, Erlemann R, Blasius S, Bongartz G, Peters PE (1993) Localization-dependent radiographic patterns in chondroblastoma. Fortschr Röntgenstr 159: 331–336 43. Wisniewski M, Toker C, Anderson PJ, Huang YP, Malis LI (1973) Chondroblastoma of the cervical spine: case report. J Neurosurg 38: 763–766