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Primary Chondroid Chordoma of the Petrous Part of the Temporal Bone
ARTICLE IN PRESS Clinical Oncology (2003) 15: 365–366
Correspondence doi:10.1016/S0936-6555(03)00151-1
Primary Chondroid Chordoma of the Petrous Part of the Temporal Bone Sir – We, present a rare case of primary chondroid chordoma of the left temporal bone, which to the best of our knowledge is the third such case of chondroid chordoma of the petrous temporal area reported in the English language literature [1,2]. A 23-year-old man presented with a 10-month history of double vision and inward deviation of his left eyeball as a consequence of left-sided VIth cranial nerve paresis. Bilateral visual acuity was evaluated as 6/9 without any scotoma. Contrast enhanced computed tomography showed calcification in the upper part of the petro-clival region, extending upwards and anteriorly into the middle cranial fossa with contrast enhancement. Magnetic resonance imaging showed a T1 hypointense lesion on the medial side of the left temporal lobe. The mass was hyperintense, with a hypointense centre on T2, and was in continuity with cavernous enhancement and petrous apex (Fig. 1). The patient underwent left orbito-zygomatic and petronal craniotomy, and the tumour was found to be located at the petrous apex, which was bony hard and contained firm grayish white areas. Histopathology showed fragments of lobulated tumour composed of large physaliferous cells, disposed in cords and embedded in abundant myxoid matrix. There were large cells with small mildly anisomorphic nuclei and abundant vacuolated bubbly cytoplasm. Bits of bony trabeculae showing infiltration by lobules of tumour were seen, along with areas of calcification and degenerative cartilage, suggestive of chondroid chordoma (Fig. 2). Immunohistochemistry was positive for pancytokeratin, S-100, alpha-antichromotrypsin, and weakly positive for epithelial membrane antigen and vimentin. Post-operatively, the patient continued to have left VIth nerve palsy, and contrast enhanced computed tomography showed residual hyperostotic areas in the region of the left petrous apex. In view of the
Fig. 1 – Contrast enhanced T1-weighted image in axial and coronal planes showing an inhomogenously enhanced mass in the left cavernous sinus region with destruction of the left lateral wall of sphenoid sinus.
Fig. 2 – Chondroid chordoma: lobules of physaliferous cells with soap bubble appearance along with cartilagenous component and focus of calcification. H&E 400. 0936-6555/03/060365+2 $30.00/0
2003 Published by Elsevier Ltd on behalf of The Royal College of Radiologists.
366
CLINICAL ONCOLOGY
residual disease, the patient was considered for post-operative radiotherapy to a dose of 66 Gy in 33 fractions over 6.5 weeks. On follow-up, the restricted left eyeball movements and diplopia had considerably improved. Chondroid chordoma is a variant of chordoma with cartilaginous component as described by Heffelfinger et al. [3]. Cranial chordomas usually occur between the fourth and fifth decade of life, whereas chondroid chordomas present in an earlier age and has a female predilection. Chordomas produce progressive cranial nerve palsies and bone destruction, which result in symptoms like pain. The two previous cases reported by Rupa et al. [2] and Hasegawa et al. [1] presented with cranial nerve palsies. The case reported by Hasegawa et al. [1] had complete tumour excision and had no local recurrence during the long-term follow-up of 10 years. However, as in the present case, Rupa et al. [2] also advocated post-operative radiotherapy in view of incomplete surgical excision of the lesion. As with chordomas, the management of chondroid chordomas warrants both surgical and radiotherapeutic interventions, as incomplete surgical excision could lead to local tumour recurrence. The addition of post-operative radiotherapy would be expected to sustain a prolonged relapse-free survival in all cases with incomplete tumour
excision [4]. P. P. MOHANTY* R. PASRICHA* N. R. DATTA* M. JAIN†
*Departments of Radiotherapy and †Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, 226014, India
References 1 Hasegawa M, Nishiyama W, Watanabe I, et al. Primary chondroid chordoma arising from the base of the temporal bone: A 10 year post-op follow up. J Laryngol Otol 1985;99:485–489. 2 Rupa V, Rajshekar V, Bhanu TS, et al. Primary chondroid chordoma of the base of the petrous temporal bone. J Laryngol Otol 1989;103:771–773. 3 Heffelfinger MJ, Dahlin DC, MacCarty CD, et al. Chordomas and cartilaginous tumours at the skull base. Cancer 1973;32:410–420. 4 Rich TA, Schiller A, Suit HD, et al. Clinical and pathological review of 48 cases of chordoma. Cancer 1985;56:182–187.
Correspondence doi:10.1016/S0936-6555(03)00151-1
Primary Chondroid Chordoma of the Petrous Part of the Temporal Bone Sir – We, present a rare case of primary chondroid chordoma of the left temporal bone, which to the best of our knowledge is the third such case of chondroid chordoma of the petrous temporal area reported in the English language literature [1,2]. A 23-year-old man presented with a 10-month history of double vision and inward deviation of his left eyeball as a consequence of left-sided VIth cranial nerve paresis. Bilateral visual acuity was evaluated as 6/9 without any scotoma. Contrast enhanced computed tomography showed calcification in the upper part of the petro-clival region, extending upwards and anteriorly into the middle cranial fossa with contrast enhancement. Magnetic resonance imaging showed a T1 hypointense lesion on the medial side of the left temporal lobe. The mass was hyperintense, with a hypointense centre on T2, and was in continuity with cavernous enhancement and petrous apex (Fig. 1). The patient underwent left orbito-zygomatic and petronal craniotomy, and the tumour was found to be located at the petrous apex, which was bony hard and contained firm grayish white areas. Histopathology showed fragments of lobulated tumour composed of large physaliferous cells, disposed in cords and embedded in abundant myxoid matrix. There were large cells with small mildly anisomorphic nuclei and abundant vacuolated bubbly cytoplasm. Bits of bony trabeculae showing infiltration by lobules of tumour were seen, along with areas of calcification and degenerative cartilage, suggestive of chondroid chordoma (Fig. 2). Immunohistochemistry was positive for pancytokeratin, S-100, alpha-antichromotrypsin, and weakly positive for epithelial membrane antigen and vimentin. Post-operatively, the patient continued to have left VIth nerve palsy, and contrast enhanced computed tomography showed residual hyperostotic areas in the region of the left petrous apex. In view of the
Fig. 1 – Contrast enhanced T1-weighted image in axial and coronal planes showing an inhomogenously enhanced mass in the left cavernous sinus region with destruction of the left lateral wall of sphenoid sinus.
Fig. 2 – Chondroid chordoma: lobules of physaliferous cells with soap bubble appearance along with cartilagenous component and focus of calcification. H&E 400. 0936-6555/03/060365+2 $30.00/0
2003 Published by Elsevier Ltd on behalf of The Royal College of Radiologists.
366
CLINICAL ONCOLOGY
residual disease, the patient was considered for post-operative radiotherapy to a dose of 66 Gy in 33 fractions over 6.5 weeks. On follow-up, the restricted left eyeball movements and diplopia had considerably improved. Chondroid chordoma is a variant of chordoma with cartilaginous component as described by Heffelfinger et al. [3]. Cranial chordomas usually occur between the fourth and fifth decade of life, whereas chondroid chordomas present in an earlier age and has a female predilection. Chordomas produce progressive cranial nerve palsies and bone destruction, which result in symptoms like pain. The two previous cases reported by Rupa et al. [2] and Hasegawa et al. [1] presented with cranial nerve palsies. The case reported by Hasegawa et al. [1] had complete tumour excision and had no local recurrence during the long-term follow-up of 10 years. However, as in the present case, Rupa et al. [2] also advocated post-operative radiotherapy in view of incomplete surgical excision of the lesion. As with chordomas, the management of chondroid chordomas warrants both surgical and radiotherapeutic interventions, as incomplete surgical excision could lead to local tumour recurrence. The addition of post-operative radiotherapy would be expected to sustain a prolonged relapse-free survival in all cases with incomplete tumour
excision [4]. P. P. MOHANTY* R. PASRICHA* N. R. DATTA* M. JAIN†
*Departments of Radiotherapy and †Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, 226014, India
References 1 Hasegawa M, Nishiyama W, Watanabe I, et al. Primary chondroid chordoma arising from the base of the temporal bone: A 10 year post-op follow up. J Laryngol Otol 1985;99:485–489. 2 Rupa V, Rajshekar V, Bhanu TS, et al. Primary chondroid chordoma of the base of the petrous temporal bone. J Laryngol Otol 1989;103:771–773. 3 Heffelfinger MJ, Dahlin DC, MacCarty CD, et al. Chordomas and cartilaginous tumours at the skull base. Cancer 1973;32:410–420. 4 Rich TA, Schiller A, Suit HD, et al. Clinical and pathological review of 48 cases of chordoma. Cancer 1985;56:182–187.