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Spinal metastasis of submandibular gland adenoid cystic carcinoma: a case report
Neoplasm
Spinal Metastasis of Submandibular Gland Adenoid Cystic Carcinoma: A Case Report Soeren Birkeland, B.A., M.D. Dronninglund Hospital, Kvaerndrup, Denmark
Birkeland S. Spinal metastasis of submandibular gland adenoid cystic carcinoma: a case report. Surg Neurol 2003;60:265– 6. BACKGROUND
Adenoid cystic carcinoma is a rare malignant tumor of the salivary glands, with pulmonary metastasis being the most common site of distant metastasis. CASE DESCRIPTION
The author reports an unusual case of intradural adenoid cystic carcinoma metastasis of the lumbar spine in a patient with recent prostate carcinoma suffering from progressive back pain. The primary submandibular tumor was resected 7 years before the appearance of the spine metastasis, and a prostatic carcinoma had been detected 1 year before. The patient died of systemically advanced disease a few months after surgical decompression. CONCLUSION
Late submandibular gland adenoid cystic carcinoma recurrence may present as an intradural lumbar spine metastasis. The case further displays difficulty in diagnosis and differentiation of metastasis in case of metachronous tumors. © 2003 Elsevier Inc. All rights reserved. KEY WORDS
Adenoid cystic carcinoma, compressive syndrome, perineural invasion, prostatic carcinoma, spinal metastasis.
denoid cystic carcinoma (ACC) is a rare malignant tumor that represents less than 1% of head and neck malignancies and 10% of salivary gland tumors [1]. ACC occurs most commonly in the major and minor salivary glands of the aerodigestive tract and skin [7]. Cases of PSA-negative staining ACC of the prostate have been reported [4]; the origin of this carcinoma is not certain, but the tumor may derive from ectopic salivary glands. Histologically, the tumor shows a cribriform arrangement. The tumor cells are small and have scant cytoplasm, but ACC has various histologic features [5]. The tumor often widely infiltrates
A
OBS Reprints not available. Correspondence to: Soeren Birkeland, Dronninglund Hospital, Vaengevej 22 DK-5772 Kvaerndrup, Denmark. Received November 12, 2002; accepted February 13, 2003. © 2003 Elsevier Inc. All rights reserved. 360 Park Avenue South, New York, NY 10010 –1710
along nerves and is difficult to eradicate. Local recurrence and metastatic spread, particularly to lung, are common but may involve bone, liver, brain [1] or quite exceptionally the spinal vertebrae (extradural metastasis) [3,6]. Treatment modalities include sole surgical resection, sole radiotherapy, or a combination of surgery and radiotherapy. However, in many studies, ACC is believed to be best managed by combined therapy [1]. The 5-year survival rates reported are 65% [1] to 87% [2], but the patient may have very late relapses leading to a 10-year survival of only 27% [2]. The incidence of newly diagnosed malignant salivary gland tumors (cases) in Denmark is 50 to 60 compared to 2,000 prostate cancers each year. In the case reported here, spinal cord compression symptoms arose in a patient with an adenoid cystic carcinoma of the past and a recent prostate cancer.
Case Description A 62-year-old man was referred to our hospital in May 2001 because of low back pain and radiation of pain into left groin. A primary submandibular gland tumor had been resected in 1994 followed by radiation therapy. The diagnosis of adenoid cystic carcinoma had been made on the biopsy obtained during surgery. Afterwards, he did well with no evidence of local recurrence. At the end of 1999, a prostate cancer had been detected by palpating a prostatic nodule on rectal examination and elevations of prostate-specific antigen (PSA). In addition, the patient had experienced sudden onset of neck and upper back pain suggesting prostate cancer bone metastases. Plain films of the spine had shown midthoracic and lumbar vertebral body collapse. The cancer had been treated surgically by orchiectomy. In May 2001, PSA level was found to be within the normal range, postorchiectomy in 1999. However, 0090-3019/03/$–see front matter doi:10.1016/S0090-3019(03)00294-5
266 Surg Neurol 2003;60:265– 6
the patient suffered from rapidly progressive back pain gradually radiating into buttock, thigh, and calf and incipient weakness in both lower extremities. Technetium-99m scintigraphy revealed increased uptake in L5. Subsequent magnetic resonance imaging (MRI) showed intraspinal tumor at level L3-4 suggesting meningioma. Chest radiograph showed an unaltered single coin lesion. Because of progression, the patient underwent acute surgery, revealing a gray-red intradural tumor mass at level L3-4. Decompression was performed as total resection was not possible without spinal cord lesion because of perineural invasion and, furthermore, the tumor was suspected of extending cranially. Histologic examination of the resected mass surprisingly showed a PSA-negative staining metastatic neoplasm identical to ACC in 1994. Postoperatively, skin tumors suggesting ACC metastases arose laterally at both upper arms. The lesions were 5-10 mm blisters with central necrosis and a surrounding zone of erythema. The skin tumors were apparently removed by radiation therapy. The course was uneventful for some months, but because of back pain recurrence, palliative irradiation therapy was performed. The patient experienced terminal rapidly progressive paresis and numbness of all extremities.
Discussion As emphasized, ACC is a difficult tumor to treat, and when metastasis occurs, the metastasis cannot be radiologically distinguished from those of other cancers. Even though the intervening prostate car-
Birkeland
cinoma did impede correct diagnosis, it is questionable if this fact had any influence on the mode of treatment recommended or the prognosis. Moreover it remains unclear if tumor progression resulted from hematogenous metastases or from direct perineural invasion from the primary submandibular gland tumor, correlating with craniocaudal pain progression. Regardless, in this case, ACC demonstrates its striking propensity for perineural invasion in a very unique way. REFERENCES 1. Chummun S, McLean NR, Kelly CG, Dawes PJ, Meikle D, Fellows S, Soames JV. Adenoid cystic carcinoma of the head and neck. Br J Plast Surg 2001;54(6):476 –80. 2. Harbo G, Grau C, Bundgaard T, Overgaard M, Elbrond O, Sogaard H, Overgaard J. Cancer of the nasal cavity and paranasal sinuses. A clinico-pathological study of 277 patients. Acta Oncol 1997;36(1):45–50. 3. Kazumoto K, Hayase N, Kurosumi M, Kishi K, Uki J, Takeda F. Multiple brain metastases from adenoid cystic carcinoma of the parotid gland. Case report and review of the literature. Surg Neurol 1998;50(5):475–9. 4. Kuhajda FP, Mann RB. Adenoid cystic carcinoma of the prostate. A case report with immunoperoxidase staining for prostate-specific acid phosphatase and prostate-specific antigen. Am J Clin Pathol 1984;81(2): 257–60. 5. Nochomovitz LE, Kahn LB. Adenoid cystic carcinoma of the salivary gland and its histologic variants. A clinicopathologic study of thirty cases. Oral Surg Oral Med Oral Pathol 1977;44(3):394 –404. 6. Riela AR, Meyer D, McCool JA, Pilcula L Jr. Metastaticadenoid cystic carcinoma of the major salivary glands presenting as a spinal cord tumor. Surg Neurol 1983; 19(4):365–8. 7. Seab JA, Graham JH. Primary cutaneous adenoid cystic carcinoma. J Am Acad Dermatol 1987;17(1):113–8.
Spinal Metastasis of Submandibular Gland Adenoid Cystic Carcinoma: A Case Report Soeren Birkeland, B.A., M.D. Dronninglund Hospital, Kvaerndrup, Denmark
Birkeland S. Spinal metastasis of submandibular gland adenoid cystic carcinoma: a case report. Surg Neurol 2003;60:265– 6. BACKGROUND
Adenoid cystic carcinoma is a rare malignant tumor of the salivary glands, with pulmonary metastasis being the most common site of distant metastasis. CASE DESCRIPTION
The author reports an unusual case of intradural adenoid cystic carcinoma metastasis of the lumbar spine in a patient with recent prostate carcinoma suffering from progressive back pain. The primary submandibular tumor was resected 7 years before the appearance of the spine metastasis, and a prostatic carcinoma had been detected 1 year before. The patient died of systemically advanced disease a few months after surgical decompression. CONCLUSION
Late submandibular gland adenoid cystic carcinoma recurrence may present as an intradural lumbar spine metastasis. The case further displays difficulty in diagnosis and differentiation of metastasis in case of metachronous tumors. © 2003 Elsevier Inc. All rights reserved. KEY WORDS
Adenoid cystic carcinoma, compressive syndrome, perineural invasion, prostatic carcinoma, spinal metastasis.
denoid cystic carcinoma (ACC) is a rare malignant tumor that represents less than 1% of head and neck malignancies and 10% of salivary gland tumors [1]. ACC occurs most commonly in the major and minor salivary glands of the aerodigestive tract and skin [7]. Cases of PSA-negative staining ACC of the prostate have been reported [4]; the origin of this carcinoma is not certain, but the tumor may derive from ectopic salivary glands. Histologically, the tumor shows a cribriform arrangement. The tumor cells are small and have scant cytoplasm, but ACC has various histologic features [5]. The tumor often widely infiltrates
A
OBS Reprints not available. Correspondence to: Soeren Birkeland, Dronninglund Hospital, Vaengevej 22 DK-5772 Kvaerndrup, Denmark. Received November 12, 2002; accepted February 13, 2003. © 2003 Elsevier Inc. All rights reserved. 360 Park Avenue South, New York, NY 10010 –1710
along nerves and is difficult to eradicate. Local recurrence and metastatic spread, particularly to lung, are common but may involve bone, liver, brain [1] or quite exceptionally the spinal vertebrae (extradural metastasis) [3,6]. Treatment modalities include sole surgical resection, sole radiotherapy, or a combination of surgery and radiotherapy. However, in many studies, ACC is believed to be best managed by combined therapy [1]. The 5-year survival rates reported are 65% [1] to 87% [2], but the patient may have very late relapses leading to a 10-year survival of only 27% [2]. The incidence of newly diagnosed malignant salivary gland tumors (cases) in Denmark is 50 to 60 compared to 2,000 prostate cancers each year. In the case reported here, spinal cord compression symptoms arose in a patient with an adenoid cystic carcinoma of the past and a recent prostate cancer.
Case Description A 62-year-old man was referred to our hospital in May 2001 because of low back pain and radiation of pain into left groin. A primary submandibular gland tumor had been resected in 1994 followed by radiation therapy. The diagnosis of adenoid cystic carcinoma had been made on the biopsy obtained during surgery. Afterwards, he did well with no evidence of local recurrence. At the end of 1999, a prostate cancer had been detected by palpating a prostatic nodule on rectal examination and elevations of prostate-specific antigen (PSA). In addition, the patient had experienced sudden onset of neck and upper back pain suggesting prostate cancer bone metastases. Plain films of the spine had shown midthoracic and lumbar vertebral body collapse. The cancer had been treated surgically by orchiectomy. In May 2001, PSA level was found to be within the normal range, postorchiectomy in 1999. However, 0090-3019/03/$–see front matter doi:10.1016/S0090-3019(03)00294-5
266 Surg Neurol 2003;60:265– 6
the patient suffered from rapidly progressive back pain gradually radiating into buttock, thigh, and calf and incipient weakness in both lower extremities. Technetium-99m scintigraphy revealed increased uptake in L5. Subsequent magnetic resonance imaging (MRI) showed intraspinal tumor at level L3-4 suggesting meningioma. Chest radiograph showed an unaltered single coin lesion. Because of progression, the patient underwent acute surgery, revealing a gray-red intradural tumor mass at level L3-4. Decompression was performed as total resection was not possible without spinal cord lesion because of perineural invasion and, furthermore, the tumor was suspected of extending cranially. Histologic examination of the resected mass surprisingly showed a PSA-negative staining metastatic neoplasm identical to ACC in 1994. Postoperatively, skin tumors suggesting ACC metastases arose laterally at both upper arms. The lesions were 5-10 mm blisters with central necrosis and a surrounding zone of erythema. The skin tumors were apparently removed by radiation therapy. The course was uneventful for some months, but because of back pain recurrence, palliative irradiation therapy was performed. The patient experienced terminal rapidly progressive paresis and numbness of all extremities.
Discussion As emphasized, ACC is a difficult tumor to treat, and when metastasis occurs, the metastasis cannot be radiologically distinguished from those of other cancers. Even though the intervening prostate car-
Birkeland
cinoma did impede correct diagnosis, it is questionable if this fact had any influence on the mode of treatment recommended or the prognosis. Moreover it remains unclear if tumor progression resulted from hematogenous metastases or from direct perineural invasion from the primary submandibular gland tumor, correlating with craniocaudal pain progression. Regardless, in this case, ACC demonstrates its striking propensity for perineural invasion in a very unique way. REFERENCES 1. Chummun S, McLean NR, Kelly CG, Dawes PJ, Meikle D, Fellows S, Soames JV. Adenoid cystic carcinoma of the head and neck. Br J Plast Surg 2001;54(6):476 –80. 2. Harbo G, Grau C, Bundgaard T, Overgaard M, Elbrond O, Sogaard H, Overgaard J. Cancer of the nasal cavity and paranasal sinuses. A clinico-pathological study of 277 patients. Acta Oncol 1997;36(1):45–50. 3. Kazumoto K, Hayase N, Kurosumi M, Kishi K, Uki J, Takeda F. Multiple brain metastases from adenoid cystic carcinoma of the parotid gland. Case report and review of the literature. Surg Neurol 1998;50(5):475–9. 4. Kuhajda FP, Mann RB. Adenoid cystic carcinoma of the prostate. A case report with immunoperoxidase staining for prostate-specific acid phosphatase and prostate-specific antigen. Am J Clin Pathol 1984;81(2): 257–60. 5. Nochomovitz LE, Kahn LB. Adenoid cystic carcinoma of the salivary gland and its histologic variants. A clinicopathologic study of thirty cases. Oral Surg Oral Med Oral Pathol 1977;44(3):394 –404. 6. Riela AR, Meyer D, McCool JA, Pilcula L Jr. Metastaticadenoid cystic carcinoma of the major salivary glands presenting as a spinal cord tumor. Surg Neurol 1983; 19(4):365–8. 7. Seab JA, Graham JH. Primary cutaneous adenoid cystic carcinoma. J Am Acad Dermatol 1987;17(1):113–8.