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0International Journal of Surgery Case Reports xxx (2014) xxx-xxx
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JOURNAL OF SURGERY
CASE REPORTS
Case report: Successful treatment of recurrent chordoma and bilateral pulmonary metastases following an 11-year disease-free period
qi Cherie P. Erkmen2'*, Richard J. Barthb, Vignesh Raman3
a Division of Thoracic Surgery, Department of Surgery, Dartmouth-Hitchcock Medical Center, One Medical Center Drive, Lebanon, NH 03756, United States b Section ofSurgical Oncology, Department ofSurgery, Dartmouth-Hitchcock Medical Center, One Medical Center Drive, Lebanon, NH 03756, United States
10 11 12
ARTICLE INFO
Article history:
Received 8 November 2013
Received in revised form 1 February 2014
Accepted 7 February 2014
Available online xxx
Keywords: Chordoma Recurrence Metastasis
ABSTRACT
INTRODUCTION: Chordomas are rare but aggressive tumors due to local recurrence and rare distant metastases. They originate commonly in the sphenooccipital and sacrococcygeal regions, and metastasize to the lungs, bone, skin, liver, and lymph nodes. They occur more frequently in men and people over the age of 40.
PRESENTATION OF CASE: A 28 year-old female presented with sacrococcygeal chordoma for which she received wide local excision and adjuvant radiation therapy. She enjoyed an unusual disease-free survival for 11 years until a routine surveillance scan of the pelvis identified local recurrence. Further work up revealed bilateral pulmonary metastases. She underwent local excision of the recurrent tumor and video-assisted thoracoscopic (VATS) wedge resection of pulmonary metastases. She also received adjuvant radiation therapy to the recurrent resection bed. Two years later, she remains free of disease and symptoms.
DISCUSSION: Chordomas are commonly insensitive to chemotherapy and radiation, making surgery the most successful therapeutic modality. However, there are few guidelines on the surveillance and treatment of recurrent chordoma. We report success with aggressive surgical resection of recurrence and metastasis as well as adjuvant radiation therapy.
CONCLUSION: The prolonged survival of our patient underscores the importance of (1) aggressive surgical resection of chordoma, whether primary, recurrent, or metastatic, with adjuvant radiation therapy, (2) minimization of surgical seeding of tumor, and (3) diligent cancer surveillance.
© 2014 The Authors. Published by Elsevier Ltd. on behalf of Surgical Associates Ltd. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/3.0/).
20 1. Introduction
21 Chordomas are locally invasive, aggressive tumors of noto-
22 chordal remnants. Chordomas have a general incidence of 0.08 per
23 100,000 people and constitute 1-4% of all bone cancers.1 They are
24 especially rare in people younger than 40 and more frequent in
25 men than women.2 The median survival is 6.29 years with a 10 year
26 survival of 39.9%.2 Most primary tumors originate in the cranium
27 (32.0%), spine (32.8%), or sacrum (29.2%).2 Extra-axial metastasis
28 occurs in 3-48% of patients.3-5 Complete surgical resection is the
29 main modality of treatment. Chordomas are frequently insensi-
30 tive to chemotherapy and radiation therapy. Local recurrence is
31 the most important indicator of poor prognosis.6 Metastasis, com-
32 monly occurring in the lungs, liver, bone, lymph nodes, and skin,
33 is often associated with local recurrence of the primary tumor.5
34 Here, we describe the clinical course and examine the outcomes
35 of our management of a 39-year-old woman who presented with
* Corresponding author. Tel.: +1 603 650 8537; fax: +1 603 650 6346. E-mail address: Cherie.P.Erkmen@Hitchcock.org (C.P. Erkmen).
multifocal metastases to the lungs and local recurrence in the 36
gluteal region 11 years after resection of a sacral chordoma. 37
2. Presentation of case 38
2.1. Primary treatment: aggressive resection with adjuvant 39
radiation 40
A 28-year-old woman presented with right buttock discom- 41
fort increasing over five years. Physical examination revealed a 42
firm, immobile mass, posterior to the rectum. MRI demonstrated 43
a 20 cm mass originating from the sacrum at the level of S5 and 44
extending into the gluteus muscles. A CT-guided biopsy of the mass 45
demonstrated chordoma. The patient underwent en bloc resection 46
of the tumor and right vertical rectus abdominus myocutaneous 47
flap for perineal reconstruction. A 6-week course of adjuvant exter- 48
nal beam radiation therapy (6000 cGy) was administered. Apart 49
from occasional fecal incontinence, the patient remained otherwise 50 asymptomatic for 11 years with no evidence of local recurrence or 51
metastasis. During this period, she underwent yearly surveillance 52
abdomen and pelvis contrast enhanced CT scans. 53
http://dx.doi.org/10.1016/j.ijscr.2014.02.005
2210-2612/© 2014 The Authors. Published by Elsevier Ltd. on behalf of Surgical Associates Ltd. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/3.0/).
2 C.P. Erkmen et ai. / International Journal of Surgery Case Reports xxx (2014) xxx-xxx
Fig. 1. Axial CT slices with arrows indicating (a) local recurrence of chordoma posterior to right gluteus maximus, (b) mass eventually identified as a lipoma in the left sartorius muscle, (c) metastatic chordoma nodule in left lower lobe of the lung, and (d) metastatic chordoma nodule in right middle lobe of the lung.
54 2.2. Wide excision of local recurrence and metastasis with pulmonary metastases demonstrating original chordoma histol- 89
55 curative intent ogy. After surgery, the patient received 4600 cGy of radiation in 90
200 cGy doses to the region of the recurrent right buttock chor- 91
56 Eleven years after her initial diagnosis, surveillance CT of the doma. 92
57 abdomen and pelvis identified a calcified soft tissue density extend-
58 ing into the right gluteus muscle and a 19 mm fatty density in the 2.3. Long-term follow-up 93
59 proximal left sartorius muscle (Fig. 1 a and b). The abdomen CT also
60 revealed pulmonary nodules (Fig. 1 c and d). A dedicated CT scan of She is free of disease and symptoms two years after her recur- 94
61 the chest subsequently identified four discrete pulmonary lesions: rence with metastases and 13 years after her initial diagnosis of 95
62 19 mm nodule in the right middle lobe, 3 mm nodule in the supe- chordoma. She has continued to undergo surveillance contrast 96
63 rior segment of the right lower lobe, 10 mm nodule in the left lower enhanced CT scans of her chest, abdomen, and pelvis every six 97
64 lobe, and a 10 mm nodule in the left upper lobe. Only the right mid- months. 98
65 dle lobe lesion was hypermetabolic by PET scan. Head MRI showed
66 no evidence of intracranial metastasis. CT guided biopsy of the right 3. Discussion 99
67 middle lobe nodule showed metastatic chordoma consistent with
68 the original sacral chordoma. We did not biopsy the suspected site 3.1. Pathological change in local recurrence 100
69 of local recurrence.
70 Given the appearance of locally recurrent disease with Dedifferentiation of chordoma has been reported rarely in local 101
71 oligometastatic disease, we endeavored to resect all suspected foci recurrence, and has been associated with poor prognosis.7-9 It is 102
72 of disease with curative intent. In an effort to minimize the num- unclear if sarcomatous transformation is due to (1) spontaneous 103
73 ber of operations due to the patient's limited financial means while change of the original chordoma, (2) irradiation-induced change, or 104
74 avoiding bilateral lung surgery at the same setting, we planned two (3) polyclonal convergence of two different cancers.9 Our patient 105
75 surgeries. The patient first underwent video assisted thoracoscopic is the first known report of dedifferentiated chordoma recurrence 106
76 surgery (VATS) removal and diagnostic biopsy of the two left-sided with differentiated chordoma pulmonary metastases. The lung 107
77 pulmonary nodules. Both left lung nodules were metastatic chor- metastases could have occurred early, before transformation of 108
78 doma resected with negative margins. A month later, the patient the initial chordoma and taken an occult, insidious course, or they 109
79 had wide local excision of right buttock mass, right sartorius mass, could have evolved from differentiated chordoma cells in a poly- 110
80 and VATS resection of two right-sided pulmonary nodules. All lung clonal tumor recurrence. The literature supports the latter scenario 111
81 specimens removed via a retrieval bag (ENDOPOUCH Specimen as local recurrence typically precedes metastasis.18 112
82 Retrieval Bag System, Ethicon, Cincinnati, OH). The right buttock
83 mass was dedifferentiated chordoma with a high-grade spindle cell 3.2. Surgery 113
84 sarcomatous element and negative margins (Fig. 2). The sartorius
85 mass was an intramuscular lipoma. The right middle lobe lesion Aggressive en bloc surgical treatment has been associated with 114
86 was chordoma with negative margins; the right lower lobe lesion decreased local recurrence rates, decreased incidence of metas- 115
87 was an intrapulmonary lymph node. The final diagnosis was local tasis, and overall improved survival.3,10 Since chordomas tend to 116
88 recurrence and dedifferentiation of sacral chordoma with bilateral be locally destructive tumors, en bloc resection may require tissue 117
Fig. 2. Pathology H&E stains of gluteal mass showing (a) chordoma cells with characteristic physaliferous cytoplasm and myxoid stroma (20x), (b) lobulated growth pattern of the tumor (4x), (c) zonal necrosis in the area of the tumor (4x), and (d) tumor invading the bone (4x).
118 reconstruction.10,11 In our patient, en bloc resection of the sacral
119 tumor with negative tissue margins and reconstruction of her per-
120 ineum led to a long disease free survival after initial treatment. We
121 therefore advocate aggressive initial surgical resection.
122 Local recurrence can originate from seeding of the tumor during
123 biopsy, resection of the primary tumor, or reconstruction.12,13 To
124 minimize the local recurrence, we recommend (1) wide resection
125 margins, (2) minimal handling of the tumor, and (3) new instru-
126 ments, and gloves be used for reconstruction and closure.13 With
127 pulmonary metastasis, we advocate a VATS wedge resection with
128 wide margins and specimen removal via a retrieval bag to prevent
129 tumor contamination of port sites.
130 3.3. Radiation therapy
131 The use of radiotherapy to treat primary and secondary chor-
132 doma is controversial, mitigated by the intolerance of adjacent
133 spinal cord and brain stem to high radiation doses.14 High dose
134 radiotherapy with surgery has been proven to favorably affect dis-
135 ease free interval.15 Conventional radiation therapy at 40-60 Gy
136 has resulted in 5-year local control of 10-40%.14,16 Our patient was
137 treated with adjuvant radiation therapy for six months following
138 initial resection and also received adjuvant radiation to the site of
139 local recurrence. While the efficacy of adjuvant radiation therapy in
140 the setting of recurrence is unknown,17 adjuvant radiation therapy
141 may be a contributor to her continued long-term survival.
142 3.4. Follow-up
143 There are poor evidence-based recommendations on chordoma
144 patient follow-up. After initial treatment, patients are followed
145 with frequent office visits in the immediate post-operative period
146 and then yearly surveillance abdomen and pelvis CT scans. Clini-
147 cal suspicion for local recurrence should be higher in patients who
148 underwent incomplete excision of their original chordoma. Any
149 new nodules, fistulas, draining sinuses, or pain and discomfort in
an area even remote from the previous excision warrant investi- 150
gation for possible recurrence. Local recurrence typically precedes 151
metastasis.18 Therefore, we recommend that patients with local 152
recurrence should undergo an evaluation for metastasis with full 153
body PET/CT scan and head MRI.The role of routine surveillance for 154
distant metastasis is unknown at this time. 155
3.5. Treatment of recurrent and metastatic disease 156
Even with recurrence and metastasis, long-term survival can be 157
achieved with successful surgical resection of all appreciable dis- 158
ease, as seen with our patient. Aggressive en bloc resection of the 159
recurrent tumor with seeding precautions is again preferred. There 160
are no reports, let alone consensus, about the treatment of pul- 161
monary metastases in chordoma. We therefore extrapolated our 162
experience with other pulmonary metastasis, electing for VATS 163
wedge resections of each of the pulmonary nodules with negative 164
margins.19,20 For metastatic chordoma to the lung, we recom- 165
mend VATS resection if (1) the primary or recurrent tumor can 166
be completely resected (2) there are no other metastases, and (3) 167
the lung metastases can be resected with ample margins without 168
compromising the patient's quality of life. Even multiple, bilateral 169
pulmonary metastases can be aggressively treated with long-term 170
success. 171
4. Conclusion 172
The treatment of chordoma demands both aggressive resection 173
and cautious attention to minimizing surgical tumor implanta- 174
tion. Adjuvant therapy is controversial but known to contribute 175
to long-term disease control and survival. Postoperative patient 176
follow-up should continue long-term; signs or symptoms consis- 177
tent with possible local recurrence or metastasis should prompt 178
a thorough work-up. In the absence of symptoms, surveillance CT 179
scans can detect potential treatable recurrence. Recurrent disease 180
C.P. Erkmen et al. / International Journal of Surgery Case Reports xxx (2014) xxx-xxx
81 can be treated aggressively with curative intent including surgery
82 and adjuvant radiation therapy.
83 Conflict of interest
84 The authors have no conflicts of interest to disclose.
85 Funding
86 None.
87 Ethical approval
88 Written informed consent was obtained from the patient for
89 publication of this case report and accompanying images. A copy
90 of the written consent is available for review by the Editor-in-Chief
91 of this journal on request
92 Author contributions
93 CPE: Study concept, data collection, data analysis, writing paper.
94 RJB: Study concept, data collection, reviewing manuscript. VR:
95 Study concept, data analysis, writing paper.
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