A report of laryngeal adenocystic carcinoma metastatic to the spleen and the role of splenectomy in the management of metastatic disease: a case report
© Murray et al; licensee BioMed Central Ltd. 2010
Received: 24 October 2009
Accepted: 6 July 2010
Published: 6 July 2010
Adenoid cystic carcinoma (ACC) of the larynx is a rare malignancy characterized by an indolent course and late pulmonary metastases. Metastases from the larynx to the spleen are an unusual event. In the present report, we discuss a patient with adenoid cystic carcinoma of the larynx metastatic to the spleen. A review of the literature did not yield any other such incidents. We review the clinical presentation and course of adenoid cystic carcinoma, as well as the role of splenectomy for metastases.
We present a case of laryngeal adenoid cystic carcinoma in a 26-year-old Caucasian man treated with total laryngectomy and ionizing radiation. He initially developed asynchronous pulmonary metastases, which were resected. Our patient subsequently presented with a symptomatic splenic lesion consistent with metastatic disease, for which he underwent laparoscopic splenectomy.
Splenectomy might be indicated for isolated metastases. A splenectomy effectively addresses symptoms and serves as a cytoreduction modality.
Adenoid cystic carcinoma (ACC) is a rare, malignant tumor, which usually originates from the minor salivary glands. The laryngeal variant, arising from the glandular components of the larynx, is extremely atypical . A review of the literature on ACC demonstrates only 15 cases reported in the past 40 years in a compressive analysis of the topic . Another review interrogated 1342 cases of laryngeal tumors and identified five cases of ACC . The management for both the laryngeal and glandular components consists of surgical resection and ionizing radiation, which is aimed to achieve local control. However, these modalities do not seem to affect mortality, which is typically the result of metastatic disease [2, 4]. Prophylactic lymph node dissection is only indicated for clinically involved nodes [5, 6]. ACC is characterized by an indolent growth pattern and late distant metastasis, most commonly to the lungs . Owing to the slow growth and indolent nature of this malignancy, survival for patients with laryngeal ACC is measured in decades [2, 5]. Thus, in contrast to other malignancies, ACC survival is typically not measured as five-year mortality, but more commonly at 10 or 20 years . Pulmonary metastasectomies have been reported for isolated lesions from ACC . Glandular ACC has a predilection for the lungs, but has also been reported to metastasize to other organs including brain, bone, liver, thyroid and spleen . The natural history of salivary gland ACC indicates that average time between diagnosis of the primary tumor and death was 60.1 months and the interval between occurrence of metastases and death was 33.0 months .
In the present discussion, we address the need to perform a splenectomy in an unusual clinical situation of a metastatic lesion from ACC. We present a review of the literature on metastatic lesions to the spleen and the role of splenectomy for their management.
A 26-year-old Caucasian man was referred to the surgical service with an enlarging splenic mass and left upper quadrant pain. The pain was described as dull with no exacerbating factors. He had no nausea or vomiting and no other systemic complaints. He had a history of laryngeal ACC that was treated with total laryngectomy and adjuvant radiation three years prior to this clinical visit. Within a year of the original diagnosis and treatment of ACC, our patient developed a single right lung metastasis for which he underwent thoracotomy with resection. This was followed by recurrence in the ipsilateral lung, which was again resected. He was started on Tarceva (erlotinib), an EGFR inhibitor used to treat non-small cell lung cancer, but the therapy was discontinued secondary to an intolerable rash.
Splenectomy for metastatic disease.
Survival in months (range)
11 dead @ 12 (5-59.5)
NED @ 28 (6-65)
31 NED @ 29.1 (6-144)
5 alive with disease
5 with disease @ 20.2 (6-72)
The present report is unique in its presentation of laryngeal ACC metastatic to the spleen. This case also illustrates the need to proceed with splenectomy for the management of symptoms and also to prevent substantial adverse outcomes that might result from further tumor involvement. Because of the indolent nature of ACC, a splenic lesion might achieve substantial growth, which if left untreated might rupture causing lethal hemorrhage or erode into the adjacent structures (i.e. diaphragm) causing significant symptoms and morbidity.
Splenic metastasis is a rare event. When it occurs, splenic metastasis is usually associated with widespread metastatic disease. Splenectomy may be considered for patients with isolated disease, patients needing cytoreduction prior to adjuvant therapy, and for those patients with symptomatic disease.
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
- Tewfik TL, Novick WH, Schipper HM: Adenoid cystic carcinoma of the larynx. J Otolaryngol. 1983, 12: 151-154.PubMedGoogle Scholar
- Moukarbel RV, Goldstein DP, O'Sullivan B, Gullane PJ, Brown DH, Wang L: Adenoid cystic carcinoma of the larynx: a 40-year experience. Head Neck. 2008, 30: 919-924. 10.1002/hed.20802.View ArticlePubMedGoogle Scholar
- Eschwege F, Cachin Y, Micheau C: Treatment of adenocarcinomas of the larynx. Can J Otolaryngol. 1975, 4: 290-292.PubMedGoogle Scholar
- Chen AM, Bucci MK, Weinberg V, Garcia J, Quivey JM, Schechter NR: Adenoid cystic carcinoma of the head and neck treated by surgery with or without postoperative radiation therapy: prognostic features of recurrence. Int J Radiat Oncol Biol Phys. 2006, 66: 152-159. 10.1016/j.ijrobp.2006.04.014.View ArticlePubMedGoogle Scholar
- Conley J, Dingman DL: Adenoid cystic carcinoma in the head and neck (cylindroma). Arch Otolaryngol. 1974, 100: 81-90.View ArticlePubMedGoogle Scholar
- Ferlito A, Caruso G: Biological behaviour of laryngeal adenoid cystic carcinoma. Therapeutic considerations. ORL J Otorhinolaryngol Relat Spec. 1983, 45: 245-256.View ArticlePubMedGoogle Scholar
- Donovan DT, Conley J: Adenoid cystic carcinoma of the subglottic region. Ann Otol Rhinol Laryngol. 1983, 92: 491-495.View ArticlePubMedGoogle Scholar
- Bobbio A, Copelli C, Ampollini L, Bianchi B, Carbognani P, Bettati S: Lung metastasis resection of adenoid cystic carcinoma of salivary glands. Eur J Cardiothorac Surg. 2008, 33: 790-793. 10.1016/j.ejcts.2007.12.057.View ArticlePubMedGoogle Scholar
- van der Wal JE, Becking AG, Snow GB, van der Waal I: Distant metastases of adenoid cystic carcinoma of the salivary glands and the value of diagnostic examinations during follow-up. Head Neck. 2002, 24: 779-783. 10.1002/hed.10126.View ArticlePubMedGoogle Scholar
- Berge T: Splenic metastases. Frequencies and patterns. Acta Pathol Microbiol Scand A. 1974, 82: 499-506.PubMedGoogle Scholar
- Sauer J, Sobolewski K, Dommisch K: Splenic metastases--not a frequent problem, but an underestimate location of metastases: epidemiology and course. J Cancer Res Clin Oncol. 2009, 135: 667-671. 10.1007/s00432-008-0502-3.View ArticlePubMedGoogle Scholar
- Lam KY, Tang V: Metastatic tumors to the spleen: a 25-year clinicopathologic study. Arch Pathol Lab Med. 2000, 124: 526-530.PubMedGoogle Scholar
- Comperat E, Bardier-Dupas A, Camparo P, Capron F, Charlotte F: Splenic metastases: clinicopathologic presentation, differential diagnosis, and pathogenesis. Arch Pathol Lab Med. 2007, 131: 965-969.PubMedGoogle Scholar
- Gemignani ML, Chi DS, Gurin CC, Curtin JP, Barakat RR: Splenectomy in recurrent epithelial ovarian cancer. Gynecol Oncol. 1999, 72: 407-410. 10.1006/gyno.1998.5141.View ArticlePubMedGoogle Scholar
- Lee SS, Morgenstern L, Phillips EH, Hiatt JR, Margulies DR: Splenectomy for splenic metastases: a changing clinical spectrum. Am Surg. 2000, 66: 837-840.PubMedGoogle Scholar
- Nicklin JL, Copeland LJ, O'Toole RV, Lewandowski GS, Vaccarello L, Havenar LP: Splenectomy as part of cytoreductive surgery for ovarian carcinoma. Gynecol Oncol. 1995, 58: 244-247. 10.1006/gyno.1995.1218.View ArticlePubMedGoogle Scholar
- de Wilt JH, McCarthy WH, Thompson JF: Surgical treatment of splenic metastases in patients with melanoma. J Am Coll Surg. 2003, 197: 38-43. 10.1016/S1072-7515(03)00381-8.View ArticlePubMedGoogle Scholar
- Sileri P, D'Ugo S, Benavoli D, Stolfi VM, Palmieri G, Mele A: Metachronous splenic metastasis from colonic carcinoma five years after surgery: a case report and literature review. South Med J. 2009, 102: 733-735.View ArticlePubMedGoogle Scholar
- Agha-Mohammadi S, Calne RY: Solitary splenic metastasis: case report and review of the literature. Am J Clin Oncol. 2001, 24: 306-310. 10.1097/00000421-200106000-00020.View ArticlePubMedGoogle Scholar
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