AJR 2005; 184:S12-S13
© American Roentgen Ray Society
Malignant Fibrous Histiocytoma of the Pharynx
Paras Lakhani1,
Stephen E. Rubesin2 and
Paul J. Zhang3
1 University of Pennsylvania School of Medicine, Philadelphia, PA 19104.
2 Department of Radiology, Hospital of the University of Pennsylvania, 3400
Spruce St., Philadelphia, PA 19104.
3 Department of Pathology and Laboratory Medicine, Hospital of the University of
Pennsylvania, Philadephia, PA 19104.
Received January 8, 2004;
accepted after revision April 16, 2004.
Address correspondence to S. E. Rubesin
(Stephen.Rubesin{at}uphs.upenn.edu).
Introduction
Malignant fibrous histiocytoma refers to a group of aggressive
soft-tissue tumors consisting of neoplastic mesenchymal cells with
fibroblastic and histiocytic phenotypes
[1]. Although malignant fibrous
histiocytoma is the most common soft-tissue sarcoma diagnosed in adults, it
rarely occurs in the pharynx. We recently encountered a malignant fibrous
histiocytoma arising in the pharynx. To our knowledge, malignant fibrous
histiocytoma involving the pharynx has not been reported in the radiology
literature. We present a patient in whom a double-contrast videotape
pharyngoesophagram revealed a polypoid mass in the posterior pharyngeal wall
that, on pathology, was diagnosed as a malignant fibrous histiocytoma.
Case Report
A 79-year-old man presented with a 2-month history of cough and throat
irritation. Two weeks before admission, the patient noted a change in his
voice and developed a nosebleed. An endoscopic examination performed by an
outside physician revealed a midpharyngeal mass. A double-contrast videotape
pharyngoesophagram showed a 4-cm polypoid mass arising in the posterior
pharyngeal wall, the tumor extending from the inferior margin of the level of
the C2 vertebral body to the upper hypopharynx
(Fig. 1A). The mass was limited
to the posterior pharyngeal wall and spared the distal hypopharynx and
cricopharyngeus. Laryngeal penetration occurred because epiglottis movement
was restricted by the posterior pharyngeal wall mass. The mass was
subsequently excised by surgery. The tumor arose from the pharyngeal wall and
extended to just below the normal squamous epithelial surface. Preoperative
cross-sectional imaging revealed a hypervascular tumor
(Fig. 1B). No phleboliths were
present to suggest a hemangioma and no fat to suggest a lipoma. No CT evidence
was seen of an extrinsic lesion invading the posterior pharyngeal wall or
lymphadenopathy, and no findings suggested a retropharyngeal abscess.
Histology of the mass showed a tumor composed of bizarre pleomorphic spindle
cells (Figs. 1C and
1D). Immunohistochemically, the
tumor stained negative for CD34, CKIT, SMA, desmin, cytokeratin AE-1/3, and
S-100. Pathologic diagnosis was malignant fibrous histiocytoma.
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Fig. 1A. —79-year-old man with malignant fibrous histiocytoma. Spot
radiograph from pharyngoesophagram obtained with patient in lateral position
reveals 4-cm polypoid mass (arrows) arising from posterior pharyngeal
wall. Tumor extends from lower oropharynx to upper hypopharynx. Tumor surface
is smooth; contour is mildly lobulated. Laryngeal vestibule, laryngeal
ventricle, and proximal trachea are coated by barium aspirated because of
inability of epiglottis to tilt.
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Fig. 1B. —79-year-old man with malignant fibrous histiocytoma. Axial
image at level of valleculae and epiglottic tip (small arrow) from
helical CT shows hypervascular mass (large arrows) arising from right
side of posterior pharyngeal wall. Mass is more vascular than adjacent
pharyngeal muscles, paraspinal muscles, or tongue. No lymphadenopathy or
phleboliths are seen.
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Fig. 1C. —79-year-old man with malignant fibrous histiocytoma.
Low-power photomicrograph shows tumor with pleomorphic features below normal
squamous epithelium of posterior pharyngeal wall. (H and E, x100)
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Almost all primary polypoid masses of the posterior oropharyngeal and
hypopharyngeal wall are squamous cell carcinomas. Radiographically and
endoscopically, the contour of this tumor was lobulated, but its mucosal
surface was smooth. Nevertheless, the endoscopist, gastrointestinal
radiologist, and neuroradiologist thought that this tumor would prove to be a
squamous cell carcinoma.
Discussion
Malignant fibrous histiocytoma usually occurs in adults between 50 and 70
years old. Approximately two thirds of those diagnosed with malignant fibrous
histiocytoma are men. The tumor most frequently occurs in the extremities,
especially in the lower extremity and thigh, but can also affect the upper
extremity and retroperitoneum. It rarely arises in the pharynx. The tumor
usually presents as a painless, enlarging mass of several months' duration,
often with no constitutional symptoms. However, retroperitoneal tumors can
often present with anorexia, malaise, weight loss, and increasing abdominal
pressure. Although there are little data on the cause of malignant fibrous
histiocytoma, it is known that some of these tumors are radiation induced. The
tumor has a local recurrence rate of 19-31%, a metastatic rate of 31-35%, and
a 5-year survival of 65-70%
[2]. The common sites for
metastasis include the lung (90%), bone (8%), and liver (1%)
[2]. Regional lymph node
metastasis is uncommon. Conservation surgery striving for negative margins
with secondary radiation therapy is considered the treatment of choice for
this disease [2,
3].
References
- Miettinen M. Diagnostic soft tissue
pathology. Philadelphia, PA: Churchill Livingstone,2003
: 197-206
- Weiss SW, Goldblum JR. Malignant fibrohistiocytic tumors. In:
Enzinger FM, Weiss SW, eds. Enzinger and Weiss's soft tissue
tumors, 4th ed. St. Louis, MO: Mosby, 2001:535
-569
- Zagars GK, Mullen JR, Pollack A. Malignant fibrous histiocytoma:
outcome and prognostic factors following conservation surgery and
radiotherapy. Int J Radiat Oncol Biol Phys1996; 34:983
-994[Medline]
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Introduction
Case Report
