- Email: [email protected]
Giant juvenile xanthogranuloma of the tongue
Giant Juvenile
Xanthogranuloma
Nina L. Shapiro, MD, David J. Malis, MD, Christopher Glenn F. Billman, MD, Donald B. Kearns, MD
of the Tongue C. Charon, MD,
Juvenile xanthogranulomas (JXGs) are rare, benign, fibrohistiocytic lesions. They usually appear as one or more cutaneous papules on the head, neck, or trunk in infants. Twelve cases of oral JXGs have been reported, four of which involved the tongue. We present a 6-year-old girl with a large tongue mass diagnosed as JXG after an excisional biopsy. Histological and immunohistochemical staining results are presented. This is the first reported case of a giant oral JXG. A review of the literature on these unusual lesions is presented, along with discussion of their differential diagnosis and key aspects of the patient’s evaluation, management, and pathological diagnosis. (Am J Otolatyngoll999;20:241-244. Copyright 0 1999 by W.B. Saunders Company)
(Editorial comment: Immunohistochemical ing is used to differentiate unusual tumors.)
stain-
CASE REPORT A previously healthy 6-year-old girl presented with an episode of acute onset oropharyngeal bleeding. Physical examination revealed a round, pedunculated, vascular mass of approximately 2 centimeters in diameter in the midline of the tongue base. The remainder of her head and neck examination was normal. Thyroid function tests were normal; a thyroid scan showed a seemingly normal thyroid gland in the central neck with no ectopic thyroid tissue. Computed tomographic scan of the neck demonstrated a well-circumscribed, round, pedicled lesion emanating from the midline tongue base, with no associated cervical lymphadenopathy (Fig 1). Examination under general anesthesia re-
From the Division of Head and Neck Surgery, UCLA School of Medicine, Los Anaeles. CA: OtolarvnaoloavHead and Neck Surgery Se&ice, ‘Brooke Army tied&l Center, San Antonio, TX; Department of Otolaryngology, Balboa Naval Hospital. San Dieao, CA: Deoartment of Pathology; and the Division of Pgdiatric Otol’aryngology, Children’s Hospital, San Diego, CA. This work was presented at the Society for Ear, Nose, and Throat Advances in Children, 25th Annual Meeting, St. Petersburg, FL, December 5, 1997. Address reprint requests to Nina L. Shapiro, MD, Pediatric Otolarvnaoloav/Division of Head and Neck Surgery, UCLA &l%ol gi Medicine, 62-158 CHS, 10833 LeConte Ave, Los Angeles, CA 90095. Copyright 0 1999 by W.B. Saunders Company 0196-0709/99/2004-0008$10.00/0
vealed that the mass was pedicled to the tongue, slightly distal to the foramen cecum, and just to the right of midline (Fig 2). The base of the pedicle was excised, incorporating a cuff of normal tongue mucosa and musculature. The patient did well postoperatively, with no airway compromise or significant dysphagia, and was discharged to go home 1 day after surgery. She was without evidence of recurrent disease at a g-month follow-up period. The resected soft tissue measured 2.4 X 2.2 X 1.8 cm. On light microscopy, the mass was composed of nests and bundles of cells, with local infiltration into the adjacent soft tissue. Cellular constituents included spindle cells, foam cells, lymphocytes, plasma cells, and occasional multinucleated giant cells. The multinucleated giant cells were distributed as nodules within a cellular matrix. The specimen also contained Touton giant cells in very small numbers. (Figs 3 and 4). Immunohistochemical staining revealed positive staining for vimentin, CD68, and CD34 in the giant, polygonal, and spindle cells. Immunohistochemical stains for Leu7, smooth muscle actin, desmin, myoglobin, epithelial membrane antigen, cytokeratin, and CDla were nonreactive. A diagnosis of giant oral juvenile xanthogranuloma (JXG) was made. DISCUSSION JXG was first reported by Adamson in 1905, who used the term congenital xanthoma multiplex to describe multiple cutaneous lesions in
American Journal of Otolaryngology, Vol20, No 4 (July-August), 1999: pp 241-244
241
242
SHAPIRO
ET AL
Fig 1. Computed tomographic image-Saggital view computed tomographic image with contrast demonstrating pedunculated soft tissue mass.
infants.’ After multiple nomenclature revisions, its modern name was introduced by Senear in 1936, and popularized by Helwig and Hackney in 195k2 Approximately 30% of all JXG lesions are present at birth, and 75%
Fig mass.
2.
lntraoperative
visualization
of tongue
base
develop during the first 9 months of life.3 It has a biphasic age distribution, peaking in infancy and in the second to third decade, with a 1 to 4:l male to female incidence.2l4 Most cutaneous lesions regress spontaneously by adolescence, leaving a flat or depressed area, which is sometimes hyperpigmented.2 JXG lesions may involve deeper tissues in 5% of patients, the eye being the most common site. Other potential noncutaneous sites include the lung, pericardium, bone, gastrointestinal tract, genitourinary tract, and central nervous system.3r5 has been retained despite The term juvenile Gartmann’s 1963 report of adults with lesions histologically consistent with JXG.4 The nomenclature can be modified clinically according to the diameter of the lesions. The papular type refers to lesions of 2 to 5 mm: the nodular type refers to lesions of 10 to 20 mm; and the macronodular, or giant type refers to lesions greater than 20 mm in diameter.” Few cases of the latter have been reported. To date, this is the first report of a giant JXG of the oral cavity. Oral JXGs are extremely rare. Adamson and Grocker8 reported lip and hard palate lesions, respectively, but without histologic documentation. The first histologically confirmed case was reported by Kjaerkeim and Stokke in 1974.g Eleven additional cases have been reported since that time.1j3,4s11-13These unencapsulated lesions occur in the vestibule, gingiva, palate, or tongue. In this location, the lesions are prone to bleeding or ulceration and may
JUVENILE
XANTHOGRANULOMA
243
Fig 3. Histological section of JXG. Low power view. Hematoxylin and eosin staining shows nonkeratiniring squamous epithelium overtying a dense cellular tumor composed of spindle cells, multinucleated giant cells, and lymphocytes. (Original magnification x40.)
cause oropharyngeal obstruction. Only one case with histologically confirmed concomitant oral and cutaneous lesions has been described.13 Unlike cutaneous lesions, which are usually diagnosed during infancy, the 12 reported patients with oral JXG lesions have a mean age at presentation of 13 years (range, 9 months to 38 years).4 Oral JXGs may be similar in their gross appearance to other more common lesions: therefore the differential diagnosis includes dental abscess, pyogenic granuloma, foreign body granuloma, fibroepithelial polyp, mucoepidermoid carcinoma, and eosinophilic granuloma.ll At the tongue base, ectopic thyroid tissue must also be considered. Accurate
diagnosis depends on histologic evaluation and immunohistochemical staining. JXGs characteristically consist of a highly cellular, nodular infiltrate of well-differentiated histiocytic or fibrohistiocytic cells. These cells are organized into sheets interspersed with acute and chronic inflammatory cells. Cytoplasmic lipid is often present, and foam cells are typically found in the specimen. Touton giant cells, typified by wreaths of multiple nuclei surrounding a glassy eosinophilic cytoplasm, are characteristic of JXG but may be absent in early lesions. In such cases, JXG may resemble eosinophilic granuloma necessitating the use of ancillary studies. The presence of Birbeck granules (Langerhan’s bodies) in the cyto-
Fig 4. Histological section of juvenile xanthogranuloma. High power view delineating multinucleated giant cells. (arrows). (Hematoxylin and Eosin; original magnification x 400.)
244
SHAPIRO
plasm, seen on electron microscopy and immunohistochemical staining for CDla, are findings that confirm the diagnosis of eosinophilic granuloma. 2*6,12Histochemical staining characteristic for JXG lesions include expression of CD45, a marker for hematopoietic cells, and CD68, a macrophage-associated antigen.4 Oral JXG is typically diagnosed after incisional or excisional biopsy, without necessarily having been considered in the differential. When the diagnosis is made from incisional biopsy, complete excision should follow. Although the typical JXG has a characteristic histologic appearance and immunohistochemical staining profile, atypical cases may be quite challenging. The diagnosis of eosinophilic granuloma and mucoepidermoid carcinoma should be excluded on completely excised tissue. This report represents the first histologically confirmed case of macronodular or giant JXG of the tongue. Preoperative radiologic evaluation to exclude other lingual masses, most notably lingual thyroid, was helpful in surgical planning. Complete resection was without perioperative complications. There has been no recurrence of the mass. ACKNOWLEDGMENT The authors acknowledge participation Cheryl Coffin in the analysis of this case.
of Dr
ET AL
REFERENCES 1. Christiensen RE Jr, Hertz RS, Cherrick HM: Intraoral juvenile xanthogranuloma. Oral Surg 45:586-590,1978 2. Tahan S, Pastel-Levy C, Bhan AK, et al: Juvenile xanthogranuloma: Clinical and pathological characterization Arch Path01 Lab Med 113:1057-1061, 1989 3. Pate1 AV, Meechan JG, Soames JV: Juvenile xanthogranuloma of the oral cavity: A case report. Int J Paediatr Dent 3:43-45,1993 4. Satow S, Zee S, Dawson KH, et al: Juvenile xanthogranuloma of the tongue. J Am Acad Dermatol33:336-339, 1995 5. Freyer DR, Kennedy R, Bostrom BC, et al: Juvenile xanthogranuloma: Forms of systemic disease and their clinical implications. J Pediatr 129:227-237, 1996 6. Magana M, Vasquez R, Fernandez-Diez J, et al: Giant congenital juvenile xanthogranuloma. Pediatr Dermatol 11:227-230,1994 7. Adamson HG: A case of congenital xanthogranuloma multiplex. Br J Dermatol 17:222,1905 8. Cracker AC: Skin xanthomas in childhood. Pediatrics 8:573-597, 1951 9. Kjaerkeim A, Stokke T: Juvenile xanthogranuloma of the oral cavity. Oral Surg Oral Med Oral Path01 38:414445,1974 10. Cohen DM, Brannon RB, Davis LD, et al: Juvenile xanthogranuloma of the oral mucosa. Oral Surg 52:513523,1981 11. Kwan CY, Min LL, Chung LC, et al: Intraoral juvenile xanthogranuloma: A case report and literature review. Oral Surg Oral Med Oral Path01 Oral Radio1 Endod 81:450-453,1996 12. Palacios J, Rodriguez-Peralto JL, Contreras F: Congenital oral juvenile xanthogranuloma: A case report. J Oral Maxillofac Surg 45:707-709,1987 13. Ossof RH, Levin DL, Esterly NB, et al: Intraoral and cutaneous juvenile xanthogranuloma. Ann Otol Rhino1 Laryngol89:268-270, 1980
Xanthogranuloma
Nina L. Shapiro, MD, David J. Malis, MD, Christopher Glenn F. Billman, MD, Donald B. Kearns, MD
of the Tongue C. Charon, MD,
Juvenile xanthogranulomas (JXGs) are rare, benign, fibrohistiocytic lesions. They usually appear as one or more cutaneous papules on the head, neck, or trunk in infants. Twelve cases of oral JXGs have been reported, four of which involved the tongue. We present a 6-year-old girl with a large tongue mass diagnosed as JXG after an excisional biopsy. Histological and immunohistochemical staining results are presented. This is the first reported case of a giant oral JXG. A review of the literature on these unusual lesions is presented, along with discussion of their differential diagnosis and key aspects of the patient’s evaluation, management, and pathological diagnosis. (Am J Otolatyngoll999;20:241-244. Copyright 0 1999 by W.B. Saunders Company)
(Editorial comment: Immunohistochemical ing is used to differentiate unusual tumors.)
stain-
CASE REPORT A previously healthy 6-year-old girl presented with an episode of acute onset oropharyngeal bleeding. Physical examination revealed a round, pedunculated, vascular mass of approximately 2 centimeters in diameter in the midline of the tongue base. The remainder of her head and neck examination was normal. Thyroid function tests were normal; a thyroid scan showed a seemingly normal thyroid gland in the central neck with no ectopic thyroid tissue. Computed tomographic scan of the neck demonstrated a well-circumscribed, round, pedicled lesion emanating from the midline tongue base, with no associated cervical lymphadenopathy (Fig 1). Examination under general anesthesia re-
From the Division of Head and Neck Surgery, UCLA School of Medicine, Los Anaeles. CA: OtolarvnaoloavHead and Neck Surgery Se&ice, ‘Brooke Army tied&l Center, San Antonio, TX; Department of Otolaryngology, Balboa Naval Hospital. San Dieao, CA: Deoartment of Pathology; and the Division of Pgdiatric Otol’aryngology, Children’s Hospital, San Diego, CA. This work was presented at the Society for Ear, Nose, and Throat Advances in Children, 25th Annual Meeting, St. Petersburg, FL, December 5, 1997. Address reprint requests to Nina L. Shapiro, MD, Pediatric Otolarvnaoloav/Division of Head and Neck Surgery, UCLA &l%ol gi Medicine, 62-158 CHS, 10833 LeConte Ave, Los Angeles, CA 90095. Copyright 0 1999 by W.B. Saunders Company 0196-0709/99/2004-0008$10.00/0
vealed that the mass was pedicled to the tongue, slightly distal to the foramen cecum, and just to the right of midline (Fig 2). The base of the pedicle was excised, incorporating a cuff of normal tongue mucosa and musculature. The patient did well postoperatively, with no airway compromise or significant dysphagia, and was discharged to go home 1 day after surgery. She was without evidence of recurrent disease at a g-month follow-up period. The resected soft tissue measured 2.4 X 2.2 X 1.8 cm. On light microscopy, the mass was composed of nests and bundles of cells, with local infiltration into the adjacent soft tissue. Cellular constituents included spindle cells, foam cells, lymphocytes, plasma cells, and occasional multinucleated giant cells. The multinucleated giant cells were distributed as nodules within a cellular matrix. The specimen also contained Touton giant cells in very small numbers. (Figs 3 and 4). Immunohistochemical staining revealed positive staining for vimentin, CD68, and CD34 in the giant, polygonal, and spindle cells. Immunohistochemical stains for Leu7, smooth muscle actin, desmin, myoglobin, epithelial membrane antigen, cytokeratin, and CDla were nonreactive. A diagnosis of giant oral juvenile xanthogranuloma (JXG) was made. DISCUSSION JXG was first reported by Adamson in 1905, who used the term congenital xanthoma multiplex to describe multiple cutaneous lesions in
American Journal of Otolaryngology, Vol20, No 4 (July-August), 1999: pp 241-244
241
242
SHAPIRO
ET AL
Fig 1. Computed tomographic image-Saggital view computed tomographic image with contrast demonstrating pedunculated soft tissue mass.
infants.’ After multiple nomenclature revisions, its modern name was introduced by Senear in 1936, and popularized by Helwig and Hackney in 195k2 Approximately 30% of all JXG lesions are present at birth, and 75%
Fig mass.
2.
lntraoperative
visualization
of tongue
base
develop during the first 9 months of life.3 It has a biphasic age distribution, peaking in infancy and in the second to third decade, with a 1 to 4:l male to female incidence.2l4 Most cutaneous lesions regress spontaneously by adolescence, leaving a flat or depressed area, which is sometimes hyperpigmented.2 JXG lesions may involve deeper tissues in 5% of patients, the eye being the most common site. Other potential noncutaneous sites include the lung, pericardium, bone, gastrointestinal tract, genitourinary tract, and central nervous system.3r5 has been retained despite The term juvenile Gartmann’s 1963 report of adults with lesions histologically consistent with JXG.4 The nomenclature can be modified clinically according to the diameter of the lesions. The papular type refers to lesions of 2 to 5 mm: the nodular type refers to lesions of 10 to 20 mm; and the macronodular, or giant type refers to lesions greater than 20 mm in diameter.” Few cases of the latter have been reported. To date, this is the first report of a giant JXG of the oral cavity. Oral JXGs are extremely rare. Adamson and Grocker8 reported lip and hard palate lesions, respectively, but without histologic documentation. The first histologically confirmed case was reported by Kjaerkeim and Stokke in 1974.g Eleven additional cases have been reported since that time.1j3,4s11-13These unencapsulated lesions occur in the vestibule, gingiva, palate, or tongue. In this location, the lesions are prone to bleeding or ulceration and may
JUVENILE
XANTHOGRANULOMA
243
Fig 3. Histological section of JXG. Low power view. Hematoxylin and eosin staining shows nonkeratiniring squamous epithelium overtying a dense cellular tumor composed of spindle cells, multinucleated giant cells, and lymphocytes. (Original magnification x40.)
cause oropharyngeal obstruction. Only one case with histologically confirmed concomitant oral and cutaneous lesions has been described.13 Unlike cutaneous lesions, which are usually diagnosed during infancy, the 12 reported patients with oral JXG lesions have a mean age at presentation of 13 years (range, 9 months to 38 years).4 Oral JXGs may be similar in their gross appearance to other more common lesions: therefore the differential diagnosis includes dental abscess, pyogenic granuloma, foreign body granuloma, fibroepithelial polyp, mucoepidermoid carcinoma, and eosinophilic granuloma.ll At the tongue base, ectopic thyroid tissue must also be considered. Accurate
diagnosis depends on histologic evaluation and immunohistochemical staining. JXGs characteristically consist of a highly cellular, nodular infiltrate of well-differentiated histiocytic or fibrohistiocytic cells. These cells are organized into sheets interspersed with acute and chronic inflammatory cells. Cytoplasmic lipid is often present, and foam cells are typically found in the specimen. Touton giant cells, typified by wreaths of multiple nuclei surrounding a glassy eosinophilic cytoplasm, are characteristic of JXG but may be absent in early lesions. In such cases, JXG may resemble eosinophilic granuloma necessitating the use of ancillary studies. The presence of Birbeck granules (Langerhan’s bodies) in the cyto-
Fig 4. Histological section of juvenile xanthogranuloma. High power view delineating multinucleated giant cells. (arrows). (Hematoxylin and Eosin; original magnification x 400.)
244
SHAPIRO
plasm, seen on electron microscopy and immunohistochemical staining for CDla, are findings that confirm the diagnosis of eosinophilic granuloma. 2*6,12Histochemical staining characteristic for JXG lesions include expression of CD45, a marker for hematopoietic cells, and CD68, a macrophage-associated antigen.4 Oral JXG is typically diagnosed after incisional or excisional biopsy, without necessarily having been considered in the differential. When the diagnosis is made from incisional biopsy, complete excision should follow. Although the typical JXG has a characteristic histologic appearance and immunohistochemical staining profile, atypical cases may be quite challenging. The diagnosis of eosinophilic granuloma and mucoepidermoid carcinoma should be excluded on completely excised tissue. This report represents the first histologically confirmed case of macronodular or giant JXG of the tongue. Preoperative radiologic evaluation to exclude other lingual masses, most notably lingual thyroid, was helpful in surgical planning. Complete resection was without perioperative complications. There has been no recurrence of the mass. ACKNOWLEDGMENT The authors acknowledge participation Cheryl Coffin in the analysis of this case.
of Dr
ET AL
REFERENCES 1. Christiensen RE Jr, Hertz RS, Cherrick HM: Intraoral juvenile xanthogranuloma. Oral Surg 45:586-590,1978 2. Tahan S, Pastel-Levy C, Bhan AK, et al: Juvenile xanthogranuloma: Clinical and pathological characterization Arch Path01 Lab Med 113:1057-1061, 1989 3. Pate1 AV, Meechan JG, Soames JV: Juvenile xanthogranuloma of the oral cavity: A case report. Int J Paediatr Dent 3:43-45,1993 4. Satow S, Zee S, Dawson KH, et al: Juvenile xanthogranuloma of the tongue. J Am Acad Dermatol33:336-339, 1995 5. Freyer DR, Kennedy R, Bostrom BC, et al: Juvenile xanthogranuloma: Forms of systemic disease and their clinical implications. J Pediatr 129:227-237, 1996 6. Magana M, Vasquez R, Fernandez-Diez J, et al: Giant congenital juvenile xanthogranuloma. Pediatr Dermatol 11:227-230,1994 7. Adamson HG: A case of congenital xanthogranuloma multiplex. Br J Dermatol 17:222,1905 8. Cracker AC: Skin xanthomas in childhood. Pediatrics 8:573-597, 1951 9. Kjaerkeim A, Stokke T: Juvenile xanthogranuloma of the oral cavity. Oral Surg Oral Med Oral Path01 38:414445,1974 10. Cohen DM, Brannon RB, Davis LD, et al: Juvenile xanthogranuloma of the oral mucosa. Oral Surg 52:513523,1981 11. Kwan CY, Min LL, Chung LC, et al: Intraoral juvenile xanthogranuloma: A case report and literature review. Oral Surg Oral Med Oral Path01 Oral Radio1 Endod 81:450-453,1996 12. Palacios J, Rodriguez-Peralto JL, Contreras F: Congenital oral juvenile xanthogranuloma: A case report. J Oral Maxillofac Surg 45:707-709,1987 13. Ossof RH, Levin DL, Esterly NB, et al: Intraoral and cutaneous juvenile xanthogranuloma. Ann Otol Rhino1 Laryngol89:268-270, 1980