Cancer Therapy Vol 6, page 321 Cancer Therapy Vol 6, 321-326, 2008
Precordial pain: an unusual primary presentation of chondrosarcoma Case Report
Vitorino Modesto dos Santos1,3,*, Fernando Henrique de Paula1,Francisco Plácido de Sousa2, Eula Leisle Braz Lima1, Natalia Wanderley Paes Barbosa3,Ricardo Carneiro Amarante3 1
Internal Medicine Thoracic Surgery Department from Armed Forces Hospital 3 Catholic University, Estrada do Contorno do Bosque s/n, Cruzeiro Novo, 70630-900, Brasilia-DF, Brazil 2
__________________________________________________________________________________ *Correspondence: Prof. Dr. Vitorino Modesto dos Santos, Catholic University Medical Course and Internal Medicine Department from Armed Forces Hospital (HFA), Brasília-DF, Brazil; Phone/Fax: #55-61-32331599; E-mail:
[email protected] Key words: chest pain, chondrosarcoma, diagnosis, neoplasm Abbreviations: chondrosarcoma, (CS), computed tomography, (CT), granulocyte-colony stimulating factor, (G-CSF) Received: 14 April 2008; Revised: 15 May 2008 Accepted: 20 May 2008; electronically published: June 2008
Summary We describe a 51-year-old male with a chondrosarcoma of the anterior chest wall detected during the investigation for the origin of an acute episode of left anterior thoracic pain. During his primary care assistance, the case was conducted as a cardiologic emergency, and the aim was to exclude eventual life-threatening cardiovascular cause for precordial pain. No abnormalities could be found by clinical standard cardiovascular diagnostic procedures, although a tender and painful mass could be easily felt on the upper left anterior thoracic area, which permitted to diagnose a well-differentiated chondrosarcoma of the chest wall. The tumor was successfully excised and the patient remains under ambulatory control. Acute chest pain is a major health problem all over the western world and constitutes diagnostic dilemmas, which result in a high number of patients in emergency departments. Although the physical examination may be unremarkable in some catastrophic conditions causing chest pain, it is often useful to detect the musculoskeletal origin of this complain.
(Gladish et al, 2002; Cakir et al, 2005). Local pain is the most frequent presenting symptom of patients with this neoplasm (Gladish et al, 2002; Ollivier et al, 2003; Hsu et al, 2006). However, precordial pains due to CS from anterior thoracic wall may rarely constitute a real diagnosis dilemma with the life-threatening cardiovascular causes of chest pain. Considering that acute thoracic pains of musculoskeletal origin may be identified by accurate examination of the muscles and joints of the neck, thoracic spine, and thorax (Stochkendahl et al, 2008), this report also aims to highlight the role of physical examination data to clear diagnoses.
I. Introduction Chondrosarcoma (CS) is a malignant tumor of slow growth rate, characterized by development of neoplastic cartilaginous tissue. The incidence of CS is higher in men around 50 years and this tumor is the third primary bone malignancy in frequency, preceded by multiple myeloma and osteosarcoma (Cakir et al, 2005; Bartalena et al, 2007). CS is more frequently found in plane bones, mainly in the pelvic and scapular girdles and in the ribs (Gladish et al, 2002; Ollivier et al, 2003; Fong et al, 2004; Cakir et al, 2005; Bartalena et al, 2007). Prognosis of CS is correlated with early diagnosis, histological type of the tumor and modality of surgery performed, in addition to availability of modern molecular biology resources (Gladish et al, 2002; Ollivier et al, 2003; Fong et al, 2004; Sakamoto et al, 2006; Chow, 2007). CS is one of the most common primary tumors of the chest wall, and is more often located in the sternum and in costochondral arches
II. Case report A 51-year-old white male was attended in Emergency Department because of an acute pain in the left hemithorax with irradiation for left scapula. In addition, he reported dyspnea, sweating and fever (39ºC) for 5 days. Previous cardiologic evaluation had ruled out pains of heart etiology. There was
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Santos et al: Precordial pain: an unusual primary presentation of chondrosarcoma antecedent of schizophrenia, diabetes mellitus, viral hepatitis C, and 60 pack-year tobacco smoking. He reported use of marijuana and cocaine 31 years ago. Medications in use were benzodiazepine, risperidone, ziprasidone, biperidene, metformin and glibenclamide. On admission, except for the BMI of 30.7 kg/m2 and the chest changes, his physical examination resulted unremarkable. Skin redness, with edema and local tenderness by digit pressure were observed over a mass which could be easily felt over the 4th and 5th left intercostal spaces in the hemiclavicular line. Both the admission electrocardiogram and controls resulted normal, and the laboratory markers were not indicative of myocardial infarction. The high levels of CPK and myoglobin were interpreted as due to some form of skeletal muscle inflammation and/or necrosis. The thorax X-ray study showed a hypodense shadow in the left apex area and opacity in the left inferior lobe (Figures 1A and 1B). The blood count showed leukocytosis associated with a high band rate (Table 1), which was considered suggestive either of infectious or inflammatory conditions. Due to the main hypotheses of pneumonia and local abscess development, he was submitted to antibiotic therapy with oxacillin and moxifloxacin, followed by improvement of the symptoms. Nevertheless, a thorax computerized tomography (CT) revealed a mass in the left anterior thoracic wall measuring 6.3 x 2.0 x 5.6 cm, which was in close contact with the pectoral musculature (Figures 1C and 1D). Thence, with the purpose of clearing the diagnosis, a CTguided needle biopsy of the mass was done. As the histology study of the biopsy samples including the immunohistochemistry data were not conclusive (Figure 2 and Table 2) and the
imaging findings were not sufficient to establish any specific diagnosis (Cakir et al, 2005), a thoracotomy was performed and the CS of anterior chest wall was disclosed involving the sternum and the fourth and fifth ribs. The wide tumor resection consisted of manubriectomy and bilateral costectomy plus left hemiclaviculectomy with free surgical margins. The histopathology study from the surgical samples revealed a well differentiated CS with few architectural changes and chondrocyte atypias (Figures 3A and 3B). The early and the late postoperative courses were uneventful and the patient became asymptomatic before his discharge from hospital. Furthermore, he was sent to oncology outpatient care and long term surveillance.
III. Discussion A. Precordial pain This 51-year-old male diabetic and obese patient, with antecedent of tobacco smoking and use of illicit drugs, presented an atypical acute precordial pain. In spite of the atypical features of the pain in the present case, the first concern of primary care physicians is the higher risk of cardiovascular origin for the chest pain (Winters and Katzen, 2006; Cakir and Blue, 2007). Therefore, the cardiovascular life-threatening causes were discarded before his admission in the clinical ward to further evaluation of possible non-cardiac etiologies (Winters and Katzen, 2006). In the vast majority of patients with acute
Figure 1. A &B. Chest x-ray study showing a hypodense shadow in the left apex area and opacity in the left inferior lobe. C & D. Images from chest CT revealing the tumor mass under the left pectoral muscle. The mass contained gas and involved the adjacent fat tissue, and was associated with a loculated left pleural effusion.
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Table 1. Laboratory data on admission and respective controls Tests Red cells Hemoglobin Hematocrit MCV MCHC White cells Platelets ESR Troponin I CPK CK-MB Myoglobin
Admission 3.85 11.4 33.4 87 34 14.5 183 77 0.02 1672 3.1 1693
Day 2 3.63 10.7 31.9 88 34 16.1 342 ND 0.01 109.7 0.5 102.8
Day 6 3.87 11.4 33.7 87 29 16.1 388 114 ND 57 0.5 103.9
Day 10 4.38 12.9 38.0 87 34 7.7 599 57 ND 56.5 ND 85.2
Normal range 4.4-6.0 x1012/mm3 11.1-16.1 g/dl 39-53 % 80-98 fl 31-36 % 4.0-11.0 x109/mm3 150-450 x109/mm3 ≤ 15 mm/h (1st hour) < 0.10 ng/ml 171.0 U/l < 3.6 ng/ml < 140.0 ng/ml
ND: not done.
Figure 2. A. Photomicrography of biopsy sample showing a reactive inflammatory infiltrate of lymphocytes and histiocytes involving connective tissue and skeletal muscle fibers (H & E, x 40). B. Photomicrography of biopsy sample showing positive CD3 lymphocytes (Immunoperoxidase, x 40). C. Photomicrography of biopsy sample showing positive CD20 lymphocytes (Immunoperoxidase, x 40). D. Photomicrography of biopsy sample showing positive CD68 lymphocytes (Immunoperoxidase, x 40).
Table 2. Immunohistochemistry data obtained from TC guided biopsy sample Antigens CD45 (LCA, leukocyte common antigen) Protein S100 Vimentin Pancytokeratin CD20 B lymphocytes (Fig. 2B) CD3 T lymphocytes (Fig. 2C) CD68 (Fig. 2D) Immunoglobulin κlight chain Immunoglobulin λlight chain
Clones PD7/26 & 2B11 V9 AE1/AE3 L26 SP7 KP1 R10-21-F3 N10/2
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Results Positive (large number of cells) Negative Positive Negative Positive (small lymphocytes) Positive (small lymphocytes) Positive (large number of macrophages) Positive (few number of cells) Positive (few number of cells)
Santos et al: Precordial pain: an unusual primary presentation of chondrosarcoma structures. The palpable mass with local pain and tenderness associated with high total leukocyte and band counts was favorable to this hypothesis. Moreover, the chest x-ray images were suggestive of pneumonia with a loculated pleural fluid, and a course of antibiotic therapy was prescribed. Notwithstanding, taking in consideration the atypical presentation precordial pain in this case, in addition to the physical examination data and the wellknown limitations of the x-ray imaging (Winters and Katzen, 2006), a CT of the thorax was performed and revealed the chest wall tumor. The thoracotomy showed that mediastinum, lungs and pleural spaces were free of tumor invasion. The excision biopsy of the tumor mass permitted us to establish the diagnosis of a low grade CS with origin in the anterior thoracic wall and expanding into the adjacent soft tissues.
B. Anterior chest wall CS Our patient is a 51-year-old male with clinical signs of inflammation and leukocytosis associated with a huge mass in the left anterior chest wall, which compressed the lung and was mimicking pulmonary affections. In adults, CS is the most frequent primary tumor of the chest wall (Ollivier et al, 2003; Cakir et al, 2005), which involves bone and soft-tissue (Chow, 2007) and is more frequently found in patients from the fourth to seventh decades, with a male predominance (Fong et al, 2004; Cakir et al, 2005; Bartalena et al, 2007). G-CSF-producing tumors associated with leukocytosis include lung cancer, malignant fibrous histiocytoma of soft tissue and the dedifferentiated CS (Sakamoto et al, 2006). The exact mechanism of leukocytosis remains unclear in the present case because the patients’ tumor was a well-differentiated CS without any dedifferentiated component, and the proinflammatory cytokine and chemokine profiles were not determined. We hypothesized that leukocytosis could be due to inflammatory mediators produced by the CS cells or tumor necrosis (Sakamoto et al, 2006), or are associated with secondary infection. In accordance with other reports, the patient’s thoracic CS was found in the sternum and in two costochondral sites (Fong et al, 2004; Cakir et al, 2005; Sakamoto et al, 2006), in association with a mass that extended into the soft tissues (Ollivier et al, 2003; Cakir et al, 2005). Patients with primary chest wall malignancies are older than those with benign tumors (Hsu et al, 2006), and the differential diagnosis of CS include neoplasms and non-tumoral conditions as: lymphoma, metastases, osteosarcoma, fibrosarcoma, neurectodermal tumor, Ewing sarcoma, histiocytoma, chondroma, chondromixoid fibroma, chondroblastoma, giant cell tumor, fibrous dysplasia, lipoma, infection, inflammation, and bone infarction (Gladish et al, 2002; Ollivier et al, 2003; Hsu et al, 2006). Worth of note, different types of sarcomas are often indistinguishable with base on radiology data (Gladish et al, 2002). Another concern can be unusual misdiagnoses with other tumors that may cause chest opacities (Jamabo and Wichendu, 2003), like observed in the present case. The treatment of choice for this low-grade CS was radical surgery, and the patient did not receive radiotherapy because all the surgical margins were free (Gladish et al, 2002; Ollivier et
Figure 3. A. Photomicrograph of tumor sample, showing well differentiated chondroid tissue with cell atypias characterized by vesicular and clustered nuclei of variable sizes, in addition to nucleus binucleation (H & E, x 250). B. Feature of the chondrocyte nucleus binucleation in detail. (H & E, x 400).
thoracic pain evaluated by primary care physicians a catastrophic condition will not be found. However, in emergency departments one must be aware about this hypothesis, because atypical presentations are common and physical examination may be unremarkable in patients with life-threatening cardiac causes of chest pain (Winters and Katzen, 2006). Worth of note, in the present case the accurate clinical history and the careful physical examination of the thorax permitted the detection of this unusual cause of precordial pain. A distinct mass was felt in the left anterior thoracic area, associated with clinical signs of local inflammatory changes. Precordial pains of non-cardiac origin are frequently complained in the clinical practice and often constitute important challenges to clinical diagnosis (Winters and Katzen, 2006; Cakir and Blue, 2007; Stochkendahl et al, 2008). Common extracardiac etiologies of precordial pain include the Tietze syndrome (costochondritis), the thoracic outlet syndrome, rib fractures and herpes zoster; and all these conditions may be strongly suspected with base on the physical examination. Although pain and tenderness are symptoms frequently reported by patients with CS from lung and chest wall (Gladish et al, 2002; Ollivier et al, 2003; Hsu et al, 2006), soft tissue and bone tumors are not enrolled among the commonest etiologies of precordial pain. Another concern was about some possible inflammatory or infectious affection of the lung and pleura with fistulous communication or direct extension to the adjacent thoracic 324
Cancer Therapy Vol 6, page 325 Morioka H, Yabe H, Kaneko S, Takaishi H, Ueda T, Watanabe M, Kobayashi K, Toyama Y (2006) Large chondrosarcoma of the rib invading the mediastinum and the spine. J Thorac Cardiovasc Surg 132, 986-987. Ollivier L, Vanel D, Leclère J (2003) Imaging of chondrosarcomas. Cancer Imaging 4, 36-38. Sakamoto A, Yamamoto H, Tanaka K, Matsuda S, Harimaya K, Oda Y, Tsuneyoshi M, Iwamoto Y (2006) Dedifferentiated chondrosarcoma with leukocytosis and elevation of serum GCSF. A case report. World J Surg Oncol 4, 37 doi:10.1186/1477-7819-4-37. Stochkendahl MJ, Christensen HW, Vach W, Hoilund-Carlsen PF, Haghfelt T, Hartvigsen J (2008) Diagnosis and treatment of musculoskeletal chest pain: design of a multi-purpose trial. BMC Musculoskelet Disord 9, 40 [Epub ahead of print]. Winters ME, Katzen SM (2006) Identifying chest pain emergencies in the primary care setting. Prim Care 33, 625642.
al, 2003; Hsu et al, 2006). In spite of the surgical difficulties caused by anatomic relationships (Fong et al, 2004), the wide excision was not followed by any adverse occurrence. Although the rate of late recurrences is not high in low-grade CS, the patient will remain on long-term oncology follow up. This report also aims to emphasize the usefulness of basic semiology methods to better clear diagnoses in primary care setting. Careful clinical history and physical examination are often useful to physician assistants accurately exclude or detect the commoner musculoskeletal causes of acute precordial pain.
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