Clinical Review & Education

Clinical Problem Solving | PATHOLOGY

A Woman With Swelling of the Posterior Pharyngeal Wall Rania Shamekh, MD; Soner Altiok, MD; Marino E. Leon, MD

A

C

B

Figure. Cytologic and histologic images from a woman with swelling of the posterior pharyngeal wall. A, Fine-needle aspiration of the tumor (Diff Quik stain, original magnification ×600). B, Immunohistochemical stains on the cell

block (AE1/AE3 immunostain, original magnification ×200). C, Resected tumor (hematoxylin-eosin, original magnification ×200).

A woman in her 50s presented with swelling of the posterior pharyngeal wall. The patient denied any associated symptoms such as pain, dysphagia, or respiratory symptoms. Her medical history included Graves disease. Her physical examination revealed a submucosal mass measuring 3.5 × 3 cm in the left posterior oropharyngeal wall extending between the soft palate and the epiglottis. The covering mucosa was intact, with no bleeding, exudates, or ulcers. Findings from her neck examination were unremarkable. Magnetic resonance imaging demonstrated fullness in the left retropharyngeal space with bone destruction of the anterior aspect of C1 and C2 vertebra. Fine-needle aspiration of the tumor revealed medium to large epithelioid cells with large central to eccentric nuclei and vacuolated cytoplasm in a metachromatic myxoid background (Figure, A). The immunohistochemical stains per-

formed on the cell block were positive for AE1/AE3 (Figure, B), CAM 5.2, S-100, and vimentin (not shown); and they were negative for p63, GFAP, desmin, and smooth muscle actin (not shown). The patient underwent a wide tumor excision. Gross inspection of the specimen revealed a nodular, reddish brown, rubbery soft tissue with areas of hemorrhage and myxomatous change. On microscopic examination, the tumor showed cords and clusters of medium to large epithelioid cells with clear to amphophilic or eosinophilic cytoplasm. The cytoplasm showed conspicuous vacuoles of varying sizes, and the nuclei were round and centrally located. These cells were embedded in a myxoid stroma (Figure, C). Immunostaining performed show tumor cells positive for AE1/AE3 and S100 (not shown). What is your diagnosis?

jamaotolaryngology.com

JAMA Otolaryngology–Head & Neck Surgery July 2014 Volume 140, Number 7

Copyright 2014 American Medical Association. All rights reserved.

Downloaded From: http://archotol.jamanetwork.com/ by a Nanyang Technological University User on 05/26/2015

671

Clinical Review & Education Clinical Problem Solving

Diagnosis Chordoma

Discussion In the current case, the formation of chords, myxoid stroma, cytoplasmic vacuoles, and positive staining for S-100 protein and cytokeratin are consistent with chordoma. The differential diagnosis in this case includes extraskeletal myxoid chondrosarcoma (EMC), myxoid liposarcoma, pleomorphic adenoma (PA), adenoid cystic carcinoma, and mucinous adenocarcinoma. Extraskeletal myxoid chondrosarcoma appears as lobular tumors with a gelatinous mucoid whitish gray cut surface. In EMC, the tumor cells are arranged in lobules and may appear as epithelioid cells arranged in cords and clusters embedded in a myxoid stroma; the cells of ECM are polygonal, spindle, stellate, or epithelioid and may show round to oval nuclei and moderate to scant cytoplasm with a high nuclear to cytoplasmic ratio. The cytoplasm may be eosinophilic with vacuolization; the vacuolization may be single, pushing the nucleus aside, resembling signet ring cells, or multiple, resembling the physaliphorous cells of chordoma. The chondrosarcoma cells may be positive for S-100 protein; however, they are negative for pancytokeratin, epithelial membrane antigen (EMA), and polyclonal carcinoembryonic antigen (CEA). Myxoid liposarcoma is rare at this location. This is a tumor that shows myxoid stroma with prominent plexiform capillary pattern; the cells are stellated and spindled with cytoplasmic vacuoles (lipoblasts). The cells in liposarcoma are not arranged in cords and lack glycogen. These cells may be positive for S-100 protein, and they are negative for cytokeratin. Pleomorphic adenoma may show a prominent myxoid and/or chondroid stroma. This tumor shows a variety of patterns with true ductal epithelial cells and myoepithelial cells. The luminal ductal cells in PA express pancytokeratin and CD117 (c-kit); the myoepithelial cells are positive for pancytokeratin, vimentin, S-100 protein, glial acidic fibrillary protein (GFAP), alpha smooth muscle actin, HHF-35 antibody, calponin, smooth muscle myosin heavy chain, and p63 immunostains. The lacunar cells in the chondroid areas are positive for S-100 protein. Adenoid cystic carcinoma shows cribriform and tubular patterns and basement membrane material. The tumor shows cells with ductular differentiation and myoepithelial cells. These latter cells ARTICLE INFORMATION

REFERENCES

Author Affiliations: College of Medicine, University of South Florida, Tampa (Shamekh); H. Lee Moffitt Cancer Center and Research Institute, Tampa, Florida (Altiok, Leon).

1. Amer HZ, Hameed M. Intraosseous benign notochordal cell tumor. Arch Pathol Lab Med. 2010; 134(2):283-288.

Corresponding Author: Marino E. Leon, MD, H. Lee Moffitt Cancer Center, Department of Anatomic Pathology, 12902 Magnolia Dr, Tampa, FL 33612 ([email protected]). Section Editor: Edward B. Stelow, MD. Published Online: June 5, 2014. doi:10.1001/jamaoto.2014.861. Conflict of Interest Disclosures: None reported.

672

show scant amount of cytoplasm and small angulated nuclei. In adenoid cystic carcinoma, the cells with ductular differentiation show expression of pancytokeratin, EMA, and CEA; and the myoepithelial cells show expression of pancytokeratin, p63, calponin, smooth muscle actin, smooth muscle myosin heavy chain, and GFAP. The tumors also may show diffuse expression of S-100 protein. Metastatic carcinoma, especially in a tumor that shows intracellular and extracellular mucin, should be considered in the differential diagnosis. Adenocarcinoma tumors may show definite glandular structures and intracytoplasmic vacuoles with content. The cells in adenocarcinoma are pleomorphic, and the nuclei exhibit irregular nuclear outlines. Adenocarcinoma cells are usually positive for cytokeratin and EMA and may express S-100 protein. Chordomas are rare tumors derived from embryonic remnants of the notochord.1-3 The incidence rate of chordoma is less than 1 per 100 000 individuals, is more common in males, and is uncommon among patients younger than 40 years.4-6 They are slowly growing malignant neoplasms that arise in the midline along the axial skeleton in the sacrococcygeal and the base of the skulls.1-3 Chordomas are divided into 3 types: conventional, chondroid, and dedifferentiated. Conventional chordomas are the most common type and show the classic morphologic characteristics. Chondroid chordomas contain both chordomatous and chondromatous components and tend to occur in the skull base region. Dedifferentiated chordomas is an aggressive transformation of the initial tumor. Grossly, chordomas present as well-circumscribed, lobulated, gelatinous tumors with hemorrhagic foci.2,3 Microscopically, chordomas show solid cords or nests of atypical epithelioid cells with vacuolated eosinophilic cytoplasm; these are the so-called physaliphorous cells. These cells are separated by fibrous tissue septa with an abundant myxoid, chondroid, or hemorrhagic matrix. The tumor cells have large atypical, central to peripheral nuclei, with mild pleomorphism and no mitosis.2,3 Imunohistochemically, chordoma cells stain positive for both epithelial and mesenchymal markers, such as vimentin, S-100 protein, cytokeratin 5/6, EMA, AE1/3, and CAM 5.2.2,3 Chordomas are locally destructive, involving bone and soft tissue and may metastasize to the lymph nodes, bone, and lung.1,3 The median survival for chordoma cases is 6.3 years, with 5- and 10-year relative survival rates of 67.6% and 39.9%, respectively.6

2. Horn KD, Fowler JC, Carrau R, Barnes EL, Rao UN. Cytokeratin immunophenotyping of an unusual cervical vertebral chordoma with extensive chondroid foci and perilaryngeal recurrence. Am J Otolaryngol. 2001;22(6):428-434.

4. Ferraresi V, Nuzzo C, Zoccali C, et al. Chordoma. BMC Cancer. 2010;10:22. 5. Hanna SA, Aston WJ, Briggs TW, Cannon SR, Saifuddin A. Sacral chordoma. Clin Orthop Relat Res. 2008;466(9):2217-2223. 6. McMaster ML, Goldstein AM, Bromley CM, Ishibe N, Parry DM. Chordoma. Cancer Causes Control. 2001;12(1):1-11.

3. Yamaguchi T, Yamato M, Saotome K. First histologically confirmed case of a classic chordoma arising in a precursor benign notochordal lesion. Skeletal Radiol. 2002;31(7):413-418.

JAMA Otolaryngology–Head & Neck Surgery July 2014 Volume 140, Number 7

Copyright 2014 American Medical Association. All rights reserved.

Downloaded From: http://archotol.jamanetwork.com/ by a Nanyang Technological University User on 05/26/2015

jamaotolaryngology.com

A woman with swelling of the posterior pharyngeal wall.

A woman with swelling of the posterior pharyngeal wall. - PDF Download Free
180KB Sizes 3 Downloads 3 Views