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Rare Submandibular Presentation of Pediatric Castleman Disease: Case Report
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PATHOLOGY
Rare Submandibular Presentation of Pediatric Castleman Disease: Case Report Jodi Hamilton, BS,* and Louis Mandel, DDSy
Q5
Q2
Castleman disease (CD), a benign lymphoproliferative disorder that affects lymph nodes, is uncommon in children, with only approximately 100 cases published. Although 23% of pediatric CD cases are found in the neck, there is no substantial reported percentage found in the salivary glands, especially the submandibular salivary gland (SMSG). A pediatric case of CD involving the SMSG is reported because of its extreme rarity. Ó 2016 Published by Elsevier Inc on behalf of the American Association of Oral and Maxillofacial Surgeons J Oral Maxillofac Surg -:1-4, 2016 Castleman disease (CD), often referred to as giant or angiofollicular lymph node hyperplasia, was first described by Castleman and Towne1 in 1954. It is a rare lymphoproliferative disorder, with a prevalence of 1 in 100,000 patients,2 characterized by a progressive lymphadenopathy that can develop anywhere in the lymphatic system. Because of its relative rarity, no large study is available to indicate the disease’s natural history. The median age is reported to be 43 years,3 with a slight female predilection.4 Its distribution in adults is reported as 60% in the thorax, 14% in the cervical area, 11% in the abdomen, and 4% in the axillary region.5,6 The entity is a very rare occurrence in children,2,5,7,8 with a distribution that differs from adults in that 33% occur in the thorax, 23% in the neck, 30% in the abdomen, and 7% in the axilla.5-7 CD is best classified into 3 histopathologic categories2,9,10: the hyaline vascular (HV), the plasma cell group, and an occasional mixed variety, with 90% being HV in type.2,6 Those cases of CD that occur in the head and neck area are almost always in the HV category.11 Lesions vary in size from 2 to 10 cm.6,12 Clinically, each histopathologic type can manifest in a unicentric or multicentric pattern. The
HV variety of CD (HVCD) is usually unicentric with a benign progression.8,9 Patients with CD of the plasma cell type tend to exhibit multicentric node involvement with aggressive growth patterns. They also usually have constitutional symptoms that can include fever, sweating, anemia, hypergammaglobulinemia, hypoalbuminemia, and an association with the POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin pigmentation) syndrome.3,6,13-15 Although the exact etiology of CD is unknown, long-term exposure to the viruses human herpesvirus-8 and human immunodeficiency virus can play a role in its development.6,10,11,15-18 In addition, overproduction of the cytokine interleukin-6 has been suggested as a causative factor.3,12,13,17 Diagnosis of CD is difficult because its clinical symptomatology is nonspecific. The HV variety classically presents as a well-circumscribed, slowly expanding, asymptomatic unicentric mass with no associated bruit or pulsation. The multicentricity of the CD plasma cell variety and its accompanying systemic manifestations facilitate its diagnosis. Involvement of a salivary gland by CD is uncommon, particularly in children. A casual review of the literature uncovered
Received from the Columbia University College of Dental Medicine
630 West 168th Street, New York, NY 10032; e-mail: Lm7@
and Salivary Gland Center, New York, NY.
Columbia.edu
*Research Assistant, Salivary Gland Center; Fourth-Year Student, Columbia University College of Dental Medicine.
Received August 26 2016 Accepted October 13 2016
yDirector, Salivary Gland Center; Associate Dean, Clinical
Ó 2016 Published by Elsevier Inc on behalf of the American Association of Oral
Professor, Department of Oral and Maxillofacial Surgery, Columbia
and Maxillofacial Surgeons
University College of Dental Medicine.
0278-2391/16/31031-X
Address
correspondence
and
reprint
requests
to
Dr
http://dx.doi.org/10.1016/j.joms.2016.10.021
Mandel: Columbia University College of Dental Medicine,
1 CRP 5.4.0 DTD YJOMS57514_proof 18 November 2016 7:16 pm CE AH
57 58 59 60 61 62 63 64 65 66 67 68 69 70 71 72 73 74 75 76 77 78 79 80 81 82 83 84 85 86 87 88 89 90 91 92 93 94 95 96 97 98 99 100 101 102 103 104 105 106 107 108 109 110 111 112
SUBMANDIBULAR PEDIATRIC CASTLEMAN DISEASE
several reports of HVCD in the parotid area of children2,6,19-25 but few pediatric cases of HVCD in the area of the submandibular salivary gland (SMSG).26-28 This case is presented because CD is uncommon in children and because of its even greater rarity in the SMSG region.
Report of Case A 12-year-old girl was seen at the Columbia University Salivary Gland Center (New York, NY) because of a slowly growing mass involving the area of the right SMSG (Fig 1). The mass had been present for at least 2 years, did not fluctuate in size, and did not cause any discomfort. Recently, growth had become a cosmetic issue and served as the impetus for care. Extraorally, the lesion was seen to occupy the area of the right SMSG and measured approximately 5 4 cm. Palpation indicated that the growth was well circumscribed, movable, firm, and painless. No cervical lymphadenopathy was present. Intraorally, no swelling of the posterior right mouth floor was evident. Simultaneous intraoral and extraoral palpation confirmed the presence of a large mass located inferior to the mylohyoid muscle in the submandibular triangle. Computed tomogram (CT) visualized the presence of a homogeneously enhancing lesion in the expected location of the right SMSG (Fig 2). A distinct right SMSG was not seen. The mass was noted to be well marginated with no evidence of local invasion. No lymphadenopathies were observed of adjacent tissues. The report stated that the lesion most
Q6
print & web 4C=FPO
113 114 115 116 117 118 119 120 121 122 123 124 125 126 127 128 129 130 131 132 133 134 135 136 137 138 139 140 141 142 143 144 145 146 147 148 149 150 151 152 153 154 155 156 157 158 159 160 161 162 163 164 165 166 167 168
2
Q4
FIGURE 1. Clinical photograph of cervical swelling. Hamilton and Mandel. Submandibular Pediatric Castleman Disease. J Oral Maxillofac Surg 2016.
likely represented a primary tumor of the SMSG, probably a pleomorphic adenoma. Other possible diagnoses included lymphoma, cat scratch disease, tuberculosis, sarcoidosis, and different benign and malignant SMSG neoplasms. Surgical removal of the circumscribed mass was facilitated by the presence of the well-developed capsule. The specimen was submitted for microscopic examination. A diagnosis of HVCD was made.
Discussion Microscopically, the pathognomonic pattern of HVCD has been defined6,13,17,18 (Fig 3). The HVCD involved lymph node exhibits many lymphoid follicles with small involuted germinal centers. The follicles, consisting of B cells, have an expanded mantle zone containing densely packed lymphocytes arranged in a concentric ring pattern. The germinal centers are atrophic and hyalinized. An increased vascularity, involving the germinal centers and the interfollicular areas, is the cause of the enhancement seen on CT. Unfortunately, HVCD has no specific diagnostic clinical features. Misdiagnosis is common, particularly because it tends to mimic a neoplasm. Because the clinical presentation is similar to other pathologic entities and its occurrence in the neck of the pediatric population, CD should be included in the differential diagnosis of all their slowly progressing cervical growths. Fine-needle aspiration biopsy of a HVCD nodule has no value because it only shows a nondiagnostic reactive lymphoid hyperplasia.6,8,11,12 These facts, combined with an infrequent occurrence, serve to impede preoperative diagnosis. It is only after microscopic examination of the surgical specimen that a definitive diagnosis of HVCD can be made. Although CD presents nonspecific clinical features, CT can be helpful in diagnosis. The enlarged culpable lymph node is isodense in relation to the adjacent soft tissue. The mass is well marginated and homogeneous and mimics the appearance of a benign neoplasm. Some help in achieving a preoperative diagnosis also is provided by the fact that the enlarged CD lymph node will enhance when contrast is used. The enhancement results from the increased vascularity of CD. In the present case, the exact location of the CD involved lymph node is open to question. Despite isolated reports of the presence of intraglandular SMSG lymph nodes,29,30 the SMSG, unlike the PG, is not Q3 known to contain intraglandular lymph nodes.31-33 The paucity of intraparenchymal SMSG lymph nodes can be explained embryologically. The lymphatic system develops after the SMSG has been encapsulated. In consequence, lymph nodes and lymphatic channels do not become entrapped within
CRP 5.4.0 DTD YJOMS57514_proof 18 November 2016 7:16 pm CE AH
Q1
169 170 171 172 173 174 175 176 177 178 179 180 181 182 183 184 185 186 187 188 189 190 191 192 193 194 195 196 197 198 199 200 201 202 203 204 205 206 207 208 209 210 211 212 213 214 215 216 217 218 219 220 221 222 223 224
3
225 226 227 228 229 230 231 232 233 234 235 236 237 238 239 240 241 242 243 244 245 246 247 248 249 250 251 252 253 254 255 256 257 258 259 260 261 262 263 264 265 266 267 268 269 270 271 272 273 274 275 276 277 278 279 280
print & web 4C=FPO
HAMILTON AND MANDEL
FIGURE 2. Contrast-enhanced computed tomogram depicts an enhanced circumscribed mass (M). Hamilton and Mandel. Submandibular Pediatric Castleman Disease. J Oral Maxillofac Surg 2016.
the parenchyma of this gland.31,34 Conversely, the embryologic development of the PG is such that the PG capsule forms after lymph node development, resulting in the known presence of intraglandular lymph nodes. No salivary gland tissue was visualized on CT or during surgery and was not observed in the microscopic study. The diseased CD lymph node probably originated within the SMSG and eventually replaced the
gland. Nevertheless, the diseased lymph node might have had an extraglandular location on the surface of the SMSG capsule. With the marked growth of this para-glandular diseased node, the SMSG can be displaced peripherally and atrophy. Therefore, the relation between the diseased node and the SMSG remains problematic. Nonetheless, involvement of the SMSG by CD in a pediatric patent is a rarity and should be reported.
CRP 5.4.0 DTD YJOMS57514_proof 18 November 2016 7:16 pm CE AH
281 282 283 284 285 286 287 288 289 290 291 292 293 294 295 296 297 298 299 300 301 302 303 304 305 306 307 308 309 310 311 312 313 314 315 316 317 318 319 320 321 322 323 324 325 326 327 328 329 330 331 332 333 334 335 336
SUBMANDIBULAR PEDIATRIC CASTLEMAN DISEASE
11.
12.
13. 14. print & web 4C=FPO
337 338 339 340 341 342 343 344 345 346 347 348 349 350 351 352 353 354 355 356 357 358 359 360 361 362 363 364 365 366 367 368 369 370 371 372 373 374 375 376 377 378 379 380 381 382 383 384 385 386 387 388 389 390 391 392
4
15.
16. 17. FIGURE 3. Microscopic view of hyaline vascular Castleman disease. The lymphoid follicles contain hyalinized germinal centers (G) that are surrounded by a concentric ring pattern of B-cell lymphocytes. Increased vascularity is present in the interfollicular area (hematoxylin and eosin stain; magnification, 100). Hamilton and Mandel. Submandibular Pediatric Castleman Disease. J Oral Maxillofac Surg 2016.
18. 19.
20. 21.
Treatment Fortunately, HVCD represents 90% of cases seen in young patients, with 90% of these being unicentric.21 The prognosis of these unicentric HVCD pediatric cases is excellent. Surgical excision of these circumscribed masses leads to full recovery.6,8,14,18 Recurrences are rare and have been attributed to incomplete removal.14 In contrast, there is no widely accepted treatment for multicentric CD. Surgery, chemotherapy steroids, radiation, and immunotherapy have had limited success.2,13,18,21,35
22.
23. 24.
25.
26.
27.
References
28.
1. Castleman B, Towne VW: Case records of the Massachusetts General Hospital: Case 4001. N Engl J Med 250:26, 1954 2. Zawawi F, Varshney R, Haegert DG, et al: Castleman’s disease: A rare finding in a pediatric neck. Int J Pediatr Otorhinolaryngol 78:370, 2014 3. Dispenzieri A, Armitage JO, Loe MJ, et al: The clinical spectrum of Castleman’s disease. Am J Hematol 87:997, 2012 4. Farruggia P, Trizzino A, Scibetta N, et al: Castleman’s disease in childhood: Report of three cases and review of the literature. Ital J Pediatr 37:50, 2011 5. Zhong L-P, Wang L-Z, Tong JI, et al: Clinical analysis of Castleman disease (hyaline vascular type) in parotid and neck region. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 109:432, 2010 6. Rabinowitz MR, Levi J, Conard K, et al: Castleman disease in the pediatric neck: A literature review. Otolaryngol Head Neck Surg 148:1028, 2013 7. Zhong L-P, Chen G-F, Zhao S-F: Cervical Castleman disease in children. Br J Oral Maxillofac Surg 42:69, 2004 8. Park JH, Lee SW, Koh YW: Castleman disease of the parotid gland in childhood: An unusual entity. Auris Nasus Larynx 35:451, 2008 9. Paratore DA, Herts BR: Castleman Disease. J Urol 194:529, 2015 10. Kawabata H, Kadowaki N, Nishikori M, et al: Clinical features and treatment of multicentric Castleman’s disease: A retrospec-
29.
30.
31.
32.
33.
34.
35.
tive study of 21 Japanese patients at a single institute. J Clin Exp Hematop 53:69, 2013 Gurbuzler L, Ceylan A, Yalmaz M, et al: Castleman’s disease of the parotid gland: A case report. Kaohsiung J Med Sci 26:444, 2010 Clain JB, Scherl S, Karle WE, et al: Castleman Disease in the parapharyngeal space: A case report and review of the literature. Head Neck Pathol 7:389, 2013 Saeed-Abdul-Rahman I, Al-Amri AM: Castleman disease. Korean J Hematol 47:163, 2012 Puram SV, Hasserjian RP, Faquin WC, et al: Castleman disease presenting in the neck: Report of a case and review of the literature. Am J Otolaryngol Head Neck Med Surg 34:239, 2013 Tartaglia F, Blasi S, Berni A, et al: Unicentric localization of Castleman ‘s disease treated with laparoscopic and traditional approach. Report of two cases. G Chir 29:399, 2008 Talat N, Schulte K-M: Castleman’s disease: Systematic analysis of 416 patients from the literature. Oncologist 16:1316, 2011 Reece B, Ord R, Papadimitriou J: Rare presentation of unicentric Castleman’s disease in the parotid gland. J Oral Maxillofac Surg 70:2114, 2012 Shah D, Darji P, Lodha S, et al: Unicentric Castleman’s disease of abdomen. Gastrointest Radiol 7:26, 2013 Harris NL, McNeely WF, Shepard J-AO, et al: Case records of the Massachusetts General Hospital: Case 19-2002. N Engl J Med 346:1989, 2002 Erdogan F, Altas S, Altas E, et al: A rare location of Castleman’s disease: Parotid region. N Z Med J 121:86, 2008 Bollig C, Moon S, Sujoy V, et al: Castleman disease of the parotid in childhood: A case report. Am J Otolaryngol Head Neck Med Surg 35:517, 2014 Kumar S, Al-Abri R, Kadir NA, et al: Castleman disease of the parotid gland in a pediatric patient: A case report. Ear Nose Throat J 93:E23, 2014 Varsak YK, Gul Z, Yucel H, et al: Pediatric Castleman disease of the parotid. J Craniofac Surg 25:1930, 2014 Delaney SW, Zhou S, Maceri D: Castleman’s disease presenting as a parotid mass in the pediatric population: A report of 2 cases. Case Rep Otolaryngol 2015:691701, 2015 Linkhom H, van der Meer G, Gruber M, et al: Castleman’s disease: An unusually young presentation resulting in delayed diagnosis of a neck mass. Int J Pediatr Otolaryngol 86:90, 2016 Sanchez-Cuellar A, de Pedro M, Martin-Granizo R, et al: Castleman disease (giant lymph node hyperplasia) in the maxillofacial region: A report of 3 cases. J Oral Maxillofac Surg 59:228, 2001 Chan SC, Ng SH, Lee KF, et al: Submandibular Castleman’s disease. Int J Clin Pract 56:314, 2002 Amine C, Abdelatif O, Nouredine E: A rare case of Castleman’s disease presenting as cervical neck mass. J Otorhinolaryngol 9:1, 2008 Chen T-C, Lo W-C, Ko J-Y, et al: Rare involvement of submandibular gland by oral squamous cell carcinoma. Head Neck 31: 877, 2009 Panda NK, Patro SK, Bakshi J, et al: Metastasis to submandibular glands in oral cavity cancers: Can we preserve the gland safely? Auris Nasus Larynx 42:322, 2015 Dhiwakar M, Ronen O, Malone J, et al: Feasibility of submandibular gland preservation in neck dissection: A prospective anatomic pathologic study. Head Neck 33:603, 2011 Takes RP, Robbins T, Woolgar JA, et al: Questionable necessity to remove the submandibular gland in neck dissection. Head Neck 33:743, 2011 Soni K, Panchal V, Kathuria B, et al: Non-Hodgkin’s lymphoma masquerading as submandibular sialadenitis. Indian J Clin Pract 25:652, 2014 Guney E, Yigitbasi OG: Functional surgical approach to the level 1 for staging early carcinoma of the lower lip. Otolaryngol Head Neck Surg 131:503, 2004 Inconetta G, Friscia M, Dell’Aversana Orabona G, et al: Castleman’s disease mimicking a parotid gland tumor: Report of a case and review of the literature. Eur Rev Med Pharmacol Sci 18:1241, 2014
CRP 5.4.0 DTD YJOMS57514_proof 18 November 2016 7:16 pm CE AH
393 394 395 396 397 398 399 400 401 402 403 404 405 406 407 408 409 410 411 412 413 414 415 416 417 418 419 420 421 422 423 424 425 426 427 428 429 430 431 432 433 434 435 436 437 438 439 440 441 442 443 444 445 446 447 448
PATHOLOGY
Rare Submandibular Presentation of Pediatric Castleman Disease: Case Report Jodi Hamilton, BS,* and Louis Mandel, DDSy
Q5
Q2
Castleman disease (CD), a benign lymphoproliferative disorder that affects lymph nodes, is uncommon in children, with only approximately 100 cases published. Although 23% of pediatric CD cases are found in the neck, there is no substantial reported percentage found in the salivary glands, especially the submandibular salivary gland (SMSG). A pediatric case of CD involving the SMSG is reported because of its extreme rarity. Ó 2016 Published by Elsevier Inc on behalf of the American Association of Oral and Maxillofacial Surgeons J Oral Maxillofac Surg -:1-4, 2016 Castleman disease (CD), often referred to as giant or angiofollicular lymph node hyperplasia, was first described by Castleman and Towne1 in 1954. It is a rare lymphoproliferative disorder, with a prevalence of 1 in 100,000 patients,2 characterized by a progressive lymphadenopathy that can develop anywhere in the lymphatic system. Because of its relative rarity, no large study is available to indicate the disease’s natural history. The median age is reported to be 43 years,3 with a slight female predilection.4 Its distribution in adults is reported as 60% in the thorax, 14% in the cervical area, 11% in the abdomen, and 4% in the axillary region.5,6 The entity is a very rare occurrence in children,2,5,7,8 with a distribution that differs from adults in that 33% occur in the thorax, 23% in the neck, 30% in the abdomen, and 7% in the axilla.5-7 CD is best classified into 3 histopathologic categories2,9,10: the hyaline vascular (HV), the plasma cell group, and an occasional mixed variety, with 90% being HV in type.2,6 Those cases of CD that occur in the head and neck area are almost always in the HV category.11 Lesions vary in size from 2 to 10 cm.6,12 Clinically, each histopathologic type can manifest in a unicentric or multicentric pattern. The
HV variety of CD (HVCD) is usually unicentric with a benign progression.8,9 Patients with CD of the plasma cell type tend to exhibit multicentric node involvement with aggressive growth patterns. They also usually have constitutional symptoms that can include fever, sweating, anemia, hypergammaglobulinemia, hypoalbuminemia, and an association with the POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin pigmentation) syndrome.3,6,13-15 Although the exact etiology of CD is unknown, long-term exposure to the viruses human herpesvirus-8 and human immunodeficiency virus can play a role in its development.6,10,11,15-18 In addition, overproduction of the cytokine interleukin-6 has been suggested as a causative factor.3,12,13,17 Diagnosis of CD is difficult because its clinical symptomatology is nonspecific. The HV variety classically presents as a well-circumscribed, slowly expanding, asymptomatic unicentric mass with no associated bruit or pulsation. The multicentricity of the CD plasma cell variety and its accompanying systemic manifestations facilitate its diagnosis. Involvement of a salivary gland by CD is uncommon, particularly in children. A casual review of the literature uncovered
Received from the Columbia University College of Dental Medicine
630 West 168th Street, New York, NY 10032; e-mail: Lm7@
and Salivary Gland Center, New York, NY.
Columbia.edu
*Research Assistant, Salivary Gland Center; Fourth-Year Student, Columbia University College of Dental Medicine.
Received August 26 2016 Accepted October 13 2016
yDirector, Salivary Gland Center; Associate Dean, Clinical
Ó 2016 Published by Elsevier Inc on behalf of the American Association of Oral
Professor, Department of Oral and Maxillofacial Surgery, Columbia
and Maxillofacial Surgeons
University College of Dental Medicine.
0278-2391/16/31031-X
Address
correspondence
and
reprint
requests
to
Dr
http://dx.doi.org/10.1016/j.joms.2016.10.021
Mandel: Columbia University College of Dental Medicine,
1 CRP 5.4.0 DTD YJOMS57514_proof 18 November 2016 7:16 pm CE AH
57 58 59 60 61 62 63 64 65 66 67 68 69 70 71 72 73 74 75 76 77 78 79 80 81 82 83 84 85 86 87 88 89 90 91 92 93 94 95 96 97 98 99 100 101 102 103 104 105 106 107 108 109 110 111 112
SUBMANDIBULAR PEDIATRIC CASTLEMAN DISEASE
several reports of HVCD in the parotid area of children2,6,19-25 but few pediatric cases of HVCD in the area of the submandibular salivary gland (SMSG).26-28 This case is presented because CD is uncommon in children and because of its even greater rarity in the SMSG region.
Report of Case A 12-year-old girl was seen at the Columbia University Salivary Gland Center (New York, NY) because of a slowly growing mass involving the area of the right SMSG (Fig 1). The mass had been present for at least 2 years, did not fluctuate in size, and did not cause any discomfort. Recently, growth had become a cosmetic issue and served as the impetus for care. Extraorally, the lesion was seen to occupy the area of the right SMSG and measured approximately 5 4 cm. Palpation indicated that the growth was well circumscribed, movable, firm, and painless. No cervical lymphadenopathy was present. Intraorally, no swelling of the posterior right mouth floor was evident. Simultaneous intraoral and extraoral palpation confirmed the presence of a large mass located inferior to the mylohyoid muscle in the submandibular triangle. Computed tomogram (CT) visualized the presence of a homogeneously enhancing lesion in the expected location of the right SMSG (Fig 2). A distinct right SMSG was not seen. The mass was noted to be well marginated with no evidence of local invasion. No lymphadenopathies were observed of adjacent tissues. The report stated that the lesion most
Q6
print & web 4C=FPO
113 114 115 116 117 118 119 120 121 122 123 124 125 126 127 128 129 130 131 132 133 134 135 136 137 138 139 140 141 142 143 144 145 146 147 148 149 150 151 152 153 154 155 156 157 158 159 160 161 162 163 164 165 166 167 168
2
Q4
FIGURE 1. Clinical photograph of cervical swelling. Hamilton and Mandel. Submandibular Pediatric Castleman Disease. J Oral Maxillofac Surg 2016.
likely represented a primary tumor of the SMSG, probably a pleomorphic adenoma. Other possible diagnoses included lymphoma, cat scratch disease, tuberculosis, sarcoidosis, and different benign and malignant SMSG neoplasms. Surgical removal of the circumscribed mass was facilitated by the presence of the well-developed capsule. The specimen was submitted for microscopic examination. A diagnosis of HVCD was made.
Discussion Microscopically, the pathognomonic pattern of HVCD has been defined6,13,17,18 (Fig 3). The HVCD involved lymph node exhibits many lymphoid follicles with small involuted germinal centers. The follicles, consisting of B cells, have an expanded mantle zone containing densely packed lymphocytes arranged in a concentric ring pattern. The germinal centers are atrophic and hyalinized. An increased vascularity, involving the germinal centers and the interfollicular areas, is the cause of the enhancement seen on CT. Unfortunately, HVCD has no specific diagnostic clinical features. Misdiagnosis is common, particularly because it tends to mimic a neoplasm. Because the clinical presentation is similar to other pathologic entities and its occurrence in the neck of the pediatric population, CD should be included in the differential diagnosis of all their slowly progressing cervical growths. Fine-needle aspiration biopsy of a HVCD nodule has no value because it only shows a nondiagnostic reactive lymphoid hyperplasia.6,8,11,12 These facts, combined with an infrequent occurrence, serve to impede preoperative diagnosis. It is only after microscopic examination of the surgical specimen that a definitive diagnosis of HVCD can be made. Although CD presents nonspecific clinical features, CT can be helpful in diagnosis. The enlarged culpable lymph node is isodense in relation to the adjacent soft tissue. The mass is well marginated and homogeneous and mimics the appearance of a benign neoplasm. Some help in achieving a preoperative diagnosis also is provided by the fact that the enlarged CD lymph node will enhance when contrast is used. The enhancement results from the increased vascularity of CD. In the present case, the exact location of the CD involved lymph node is open to question. Despite isolated reports of the presence of intraglandular SMSG lymph nodes,29,30 the SMSG, unlike the PG, is not Q3 known to contain intraglandular lymph nodes.31-33 The paucity of intraparenchymal SMSG lymph nodes can be explained embryologically. The lymphatic system develops after the SMSG has been encapsulated. In consequence, lymph nodes and lymphatic channels do not become entrapped within
CRP 5.4.0 DTD YJOMS57514_proof 18 November 2016 7:16 pm CE AH
Q1
169 170 171 172 173 174 175 176 177 178 179 180 181 182 183 184 185 186 187 188 189 190 191 192 193 194 195 196 197 198 199 200 201 202 203 204 205 206 207 208 209 210 211 212 213 214 215 216 217 218 219 220 221 222 223 224
3
225 226 227 228 229 230 231 232 233 234 235 236 237 238 239 240 241 242 243 244 245 246 247 248 249 250 251 252 253 254 255 256 257 258 259 260 261 262 263 264 265 266 267 268 269 270 271 272 273 274 275 276 277 278 279 280
print & web 4C=FPO
HAMILTON AND MANDEL
FIGURE 2. Contrast-enhanced computed tomogram depicts an enhanced circumscribed mass (M). Hamilton and Mandel. Submandibular Pediatric Castleman Disease. J Oral Maxillofac Surg 2016.
the parenchyma of this gland.31,34 Conversely, the embryologic development of the PG is such that the PG capsule forms after lymph node development, resulting in the known presence of intraglandular lymph nodes. No salivary gland tissue was visualized on CT or during surgery and was not observed in the microscopic study. The diseased CD lymph node probably originated within the SMSG and eventually replaced the
gland. Nevertheless, the diseased lymph node might have had an extraglandular location on the surface of the SMSG capsule. With the marked growth of this para-glandular diseased node, the SMSG can be displaced peripherally and atrophy. Therefore, the relation between the diseased node and the SMSG remains problematic. Nonetheless, involvement of the SMSG by CD in a pediatric patent is a rarity and should be reported.
CRP 5.4.0 DTD YJOMS57514_proof 18 November 2016 7:16 pm CE AH
281 282 283 284 285 286 287 288 289 290 291 292 293 294 295 296 297 298 299 300 301 302 303 304 305 306 307 308 309 310 311 312 313 314 315 316 317 318 319 320 321 322 323 324 325 326 327 328 329 330 331 332 333 334 335 336
SUBMANDIBULAR PEDIATRIC CASTLEMAN DISEASE
11.
12.
13. 14. print & web 4C=FPO
337 338 339 340 341 342 343 344 345 346 347 348 349 350 351 352 353 354 355 356 357 358 359 360 361 362 363 364 365 366 367 368 369 370 371 372 373 374 375 376 377 378 379 380 381 382 383 384 385 386 387 388 389 390 391 392
4
15.
16. 17. FIGURE 3. Microscopic view of hyaline vascular Castleman disease. The lymphoid follicles contain hyalinized germinal centers (G) that are surrounded by a concentric ring pattern of B-cell lymphocytes. Increased vascularity is present in the interfollicular area (hematoxylin and eosin stain; magnification, 100). Hamilton and Mandel. Submandibular Pediatric Castleman Disease. J Oral Maxillofac Surg 2016.
18. 19.
20. 21.
Treatment Fortunately, HVCD represents 90% of cases seen in young patients, with 90% of these being unicentric.21 The prognosis of these unicentric HVCD pediatric cases is excellent. Surgical excision of these circumscribed masses leads to full recovery.6,8,14,18 Recurrences are rare and have been attributed to incomplete removal.14 In contrast, there is no widely accepted treatment for multicentric CD. Surgery, chemotherapy steroids, radiation, and immunotherapy have had limited success.2,13,18,21,35
22.
23. 24.
25.
26.
27.
References
28.
1. Castleman B, Towne VW: Case records of the Massachusetts General Hospital: Case 4001. N Engl J Med 250:26, 1954 2. Zawawi F, Varshney R, Haegert DG, et al: Castleman’s disease: A rare finding in a pediatric neck. Int J Pediatr Otorhinolaryngol 78:370, 2014 3. Dispenzieri A, Armitage JO, Loe MJ, et al: The clinical spectrum of Castleman’s disease. Am J Hematol 87:997, 2012 4. Farruggia P, Trizzino A, Scibetta N, et al: Castleman’s disease in childhood: Report of three cases and review of the literature. Ital J Pediatr 37:50, 2011 5. Zhong L-P, Wang L-Z, Tong JI, et al: Clinical analysis of Castleman disease (hyaline vascular type) in parotid and neck region. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 109:432, 2010 6. Rabinowitz MR, Levi J, Conard K, et al: Castleman disease in the pediatric neck: A literature review. Otolaryngol Head Neck Surg 148:1028, 2013 7. Zhong L-P, Chen G-F, Zhao S-F: Cervical Castleman disease in children. Br J Oral Maxillofac Surg 42:69, 2004 8. Park JH, Lee SW, Koh YW: Castleman disease of the parotid gland in childhood: An unusual entity. Auris Nasus Larynx 35:451, 2008 9. Paratore DA, Herts BR: Castleman Disease. J Urol 194:529, 2015 10. Kawabata H, Kadowaki N, Nishikori M, et al: Clinical features and treatment of multicentric Castleman’s disease: A retrospec-
29.
30.
31.
32.
33.
34.
35.
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