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Pathology Quiz Case 2: Diagnosis

Pathology Quiz Case 2: Diagnosis Diagnosis: Laryngeal giant cell tumor (LGCT) Giant cell tumors are benign neoplasms that account for 5% of all primary bone tumors. They are most often seen in the metaphysical-epiphysical area of the long bones, and the most frequent locations are the distal femur, proximal tibia, and distal radius. The peak incidence is between 20 and 40 years of age.1 Two percent of all GCTs are found in the head and neck region, particularly in the sphenoidal, ethmoidal, and temporal bones.2,3 The laryngeal form is very rare. The first case of a LGCT was reported in 1940, and, to our knowledge, only 31 histopathologically confirmed cases have been published in the literature.3,4 The average age at LGCT diagnosis is 42 years (range, 23-60 years), and patients present with complaints of dysphonia, palpable masses in the head and neck region, dysphagia, sore throat, or airway obstruction. Altough GCTs of the long bones show a slight female predilection in some series, LGCTs have a clear male predilection.1,3 The most common location of LGCTs is the thyroid cartilage, followed by the cartilage of the cricoid and epiglottis. The tumors arise most often after secondary ossification of the laryngeal skeleton.3,5 Although LGCTs are benign, they can cause significant morbidity because of their size and local infiltration. Radiologic investigation includes computed tomography and magnetic resonance imaging to demonstrate the origin, extension, and local infiltration of the tumor. Fluid levels within the tumor are indicative of secondary aneurysmal bone cyst (ABC) formation. Because we had no reason to consider this rare diagnosis, preoperative imaging was not performed in the present case. The microscopic laryngoscopy was a diagnostic procedure. Clinically, LGCTs may resemble malignant neoplasms because of the sometimes rapid growth and large size. Other laryngeal neoplasms that contain giant cells must be exluded. Among them are giant cell reparative granuloma, brown tumor, osteoblastoma, chondroblastoma, nonossifying fibroma, ABC, osteosarcoma, and spindle cell carcinoma.5,6 Histopathologic interpretation is necessary to exclude LGCTs from these other tumors and is important because LGCTs require a more conservative therapy than most of these neoplasms. The diagnosis of a GCT is made by a combination of a large number of uniformly dispersed giant cells containing many nuclei that are histologically indistinguishable from the nuclei of the mononuclear cells of the stroma surrounding the giant cells. This feature can exclude GCTs from other lesions in the differential diagnosis.1,3,5 The mononuclear cells are predominantly round, oval, or polygonal, and they may resemble normal histiocytes. The stroma of most GCTs is richly vascularized, containing thin-walled capillaries that often have small areas of hemorrhage. Furthermore, GCTs are frequently associated with secondary ABC formation, but they also contain solid areas, in contrast to primary ABC formation.3,5 Cytologic atypia should be absent, thereby excluding a malignant bone-forming tumor.5 Surgery is the treatment of choice to obtain complete local control. The following surgical approaches were used in previous cases: laryngofissure (n = 4), partial laryngectomy (n = 11), and laryngectomy (n = 10).3 In 9 cases, radiotherapy was used, either as monotherapy or as adjuvant to surgery. Because of the benign nature of LGCTs and the potential malignant transformation after radiation therapy, it is preferable not to use this treatment modality for LGCTs. The long-term prognosis after therapy is good, and, to our knowledge, no previous patients have developed recurrences or metastases.3 However, reduced voice quality is a frequent related morbidity after therapy, especially after (partial) laryngectomy.5 The present case is remarkable because our patient presented with a LGCT at such an early stage that, in contrary to the treatment modalities in the literature, a simple (nonradical) excision of the tumor was sufficient to cure the patient, with no morbidity after treatment. In conclusion, primary LGCTs are benign neoplasms that are exceedingly rare and can cause significant morbidity because of size and local infiltration. Conservative surgery with complete tumor removal is the treatment of first choice, with excellent long-term prognosis. Return to Quiz Case. Submissions Residents and fellows in otolaryngology are invited to submit quiz cases for this section and to write letters commenting on cases presented. Quiz cases should follow the patterns established. See Instructions for Authors (jamaoto.com). Material for CLINICAL PROBLEM SOLVING: PATHOLOGY should be submitted electronically via the online submission and review system at http://manuscripts.jamaoto.com. Reprints are not available from the authors. References 1. Turcotte RE. Giant cell tumor of bone. Orthop Clin North Am. 2006;37(1):35-5116311110PubMedGoogle ScholarCrossref 2. Hinni ML. Giant cell tumor of the larynx. Ann Otol Rhinol Laryngol. 2000;109(1):63-6610651415PubMedGoogle Scholar 3. Nishimura K, Satoh T, Maesawa C, Ishijima K, Sato H. Giant cell tumor of the larynx: a case report and review of the literature. Am J Otolaryngol. 2007;28(6):436-44017980781PubMedGoogle ScholarCrossref 4. Shi ZP, Lee JC, Wang CH, Lin YS. Giant cell tumor of the larynx. Otolaryngol Head Neck Surg. 2008;138(1):117-11818165007PubMedGoogle ScholarCrossref 5. Wieneke JA, Gannon FH, Heffner DK, Thompson LD. Giant cell tumor of the larynx: a clinicopathologic series of eight cases and a review of the literature. Mod Pathol. 2001;14(12):1209-121511743042PubMedGoogle ScholarCrossref 6. Werner JA, Harms D, Beigel A. Giant cell tumor of the larynx: case report and review of the literature. Head Neck. 1997;19(2):153-1579059875PubMedGoogle ScholarCrossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png JAMA Otolaryngology - Head & Neck Surgery American Medical Association

Pathology Quiz Case 2: Diagnosis

JAMA Otolaryngology - Head & Neck Surgery , Volume 139 (1) – Jan 1, 2013

Pathology Quiz Case 2: Diagnosis

Abstract

Diagnosis: Laryngeal giant cell tumor (LGCT) Giant cell tumors are benign neoplasms that account for 5% of all primary bone tumors. They are most often seen in the metaphysical-epiphysical area of the long bones, and the most frequent locations are the distal femur, proximal tibia, and distal radius. The peak incidence is between 20 and 40 years of age.1 Two percent of all GCTs are found in the head and neck region, particularly in the sphenoidal, ethmoidal, and temporal bones.2,3 The...
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References (6)

Publisher
American Medical Association
Copyright
Copyright © 2013 American Medical Association. All Rights Reserved.
ISSN
2168-6181
eISSN
2168-619X
DOI
10.1001/jamaoto.2013.1145b
Publisher site
See Article on Publisher Site

Abstract

Diagnosis: Laryngeal giant cell tumor (LGCT) Giant cell tumors are benign neoplasms that account for 5% of all primary bone tumors. They are most often seen in the metaphysical-epiphysical area of the long bones, and the most frequent locations are the distal femur, proximal tibia, and distal radius. The peak incidence is between 20 and 40 years of age.1 Two percent of all GCTs are found in the head and neck region, particularly in the sphenoidal, ethmoidal, and temporal bones.2,3 The laryngeal form is very rare. The first case of a LGCT was reported in 1940, and, to our knowledge, only 31 histopathologically confirmed cases have been published in the literature.3,4 The average age at LGCT diagnosis is 42 years (range, 23-60 years), and patients present with complaints of dysphonia, palpable masses in the head and neck region, dysphagia, sore throat, or airway obstruction. Altough GCTs of the long bones show a slight female predilection in some series, LGCTs have a clear male predilection.1,3 The most common location of LGCTs is the thyroid cartilage, followed by the cartilage of the cricoid and epiglottis. The tumors arise most often after secondary ossification of the laryngeal skeleton.3,5 Although LGCTs are benign, they can cause significant morbidity because of their size and local infiltration. Radiologic investigation includes computed tomography and magnetic resonance imaging to demonstrate the origin, extension, and local infiltration of the tumor. Fluid levels within the tumor are indicative of secondary aneurysmal bone cyst (ABC) formation. Because we had no reason to consider this rare diagnosis, preoperative imaging was not performed in the present case. The microscopic laryngoscopy was a diagnostic procedure. Clinically, LGCTs may resemble malignant neoplasms because of the sometimes rapid growth and large size. Other laryngeal neoplasms that contain giant cells must be exluded. Among them are giant cell reparative granuloma, brown tumor, osteoblastoma, chondroblastoma, nonossifying fibroma, ABC, osteosarcoma, and spindle cell carcinoma.5,6 Histopathologic interpretation is necessary to exclude LGCTs from these other tumors and is important because LGCTs require a more conservative therapy than most of these neoplasms. The diagnosis of a GCT is made by a combination of a large number of uniformly dispersed giant cells containing many nuclei that are histologically indistinguishable from the nuclei of the mononuclear cells of the stroma surrounding the giant cells. This feature can exclude GCTs from other lesions in the differential diagnosis.1,3,5 The mononuclear cells are predominantly round, oval, or polygonal, and they may resemble normal histiocytes. The stroma of most GCTs is richly vascularized, containing thin-walled capillaries that often have small areas of hemorrhage. Furthermore, GCTs are frequently associated with secondary ABC formation, but they also contain solid areas, in contrast to primary ABC formation.3,5 Cytologic atypia should be absent, thereby excluding a malignant bone-forming tumor.5 Surgery is the treatment of choice to obtain complete local control. The following surgical approaches were used in previous cases: laryngofissure (n = 4), partial laryngectomy (n = 11), and laryngectomy (n = 10).3 In 9 cases, radiotherapy was used, either as monotherapy or as adjuvant to surgery. Because of the benign nature of LGCTs and the potential malignant transformation after radiation therapy, it is preferable not to use this treatment modality for LGCTs. The long-term prognosis after therapy is good, and, to our knowledge, no previous patients have developed recurrences or metastases.3 However, reduced voice quality is a frequent related morbidity after therapy, especially after (partial) laryngectomy.5 The present case is remarkable because our patient presented with a LGCT at such an early stage that, in contrary to the treatment modalities in the literature, a simple (nonradical) excision of the tumor was sufficient to cure the patient, with no morbidity after treatment. In conclusion, primary LGCTs are benign neoplasms that are exceedingly rare and can cause significant morbidity because of size and local infiltration. Conservative surgery with complete tumor removal is the treatment of first choice, with excellent long-term prognosis. Return to Quiz Case. Submissions Residents and fellows in otolaryngology are invited to submit quiz cases for this section and to write letters commenting on cases presented. Quiz cases should follow the patterns established. See Instructions for Authors (jamaoto.com). Material for CLINICAL PROBLEM SOLVING: PATHOLOGY should be submitted electronically via the online submission and review system at http://manuscripts.jamaoto.com. Reprints are not available from the authors. References 1. Turcotte RE. Giant cell tumor of bone. Orthop Clin North Am. 2006;37(1):35-5116311110PubMedGoogle ScholarCrossref 2. Hinni ML. Giant cell tumor of the larynx. Ann Otol Rhinol Laryngol. 2000;109(1):63-6610651415PubMedGoogle Scholar 3. Nishimura K, Satoh T, Maesawa C, Ishijima K, Sato H. Giant cell tumor of the larynx: a case report and review of the literature. Am J Otolaryngol. 2007;28(6):436-44017980781PubMedGoogle ScholarCrossref 4. Shi ZP, Lee JC, Wang CH, Lin YS. Giant cell tumor of the larynx. Otolaryngol Head Neck Surg. 2008;138(1):117-11818165007PubMedGoogle ScholarCrossref 5. Wieneke JA, Gannon FH, Heffner DK, Thompson LD. Giant cell tumor of the larynx: a clinicopathologic series of eight cases and a review of the literature. Mod Pathol. 2001;14(12):1209-121511743042PubMedGoogle ScholarCrossref 6. Werner JA, Harms D, Beigel A. Giant cell tumor of the larynx: case report and review of the literature. Head Neck. 1997;19(2):153-1579059875PubMedGoogle ScholarCrossref

Journal

JAMA Otolaryngology - Head & Neck SurgeryAmerican Medical Association

Published: Jan 1, 2013

Keywords: neoplasms,larynx

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