- Email: [email protected]
Follicular dendritic cell sarcoma of the nasopharynx
Int. J. Oral Maxillofac. Surg. 2012; 41: 218–220 doi:10.1016/j.ijom.2011.06.024, available online at http://www.sciencedirect.com
Case Report Head and Neck Oncology
Follicular dendritic cell sarcoma of the nasopharynx
B. Karabulut1, K. S. Orhan2, Y. Guldiken2, O. Dogan3 1
Department of Otolaryngology, Ardahan State Hospital, Turkey; 2Department of Otolaryngology Head and Neck Surgery, Istanbul University, Istanbul Medical Faculty, Turkey; 3Department of Pathology, Istanbul University, Istanbul Medical Faculty, Turkey
B. Karabulut, K. S. Orhan, Y. Guldiken, O. Dogan: Follicular dendritic cell sarcoma of the nasopharynx. Int. J. Oral Maxillofac. Surg. 2012; 41: 218–220. # 2011 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved. Abstract. Follicular dendritic cell (FDC) sarcomas of the nasopharynx are rare tumours; only seven cases have been reported in the English language medical literature. The authors present an eighth case, which occurred in a 70-year-old woman whose main complaint was nasal obstruction. It has been more than 10 years since FDC sarcoma was reported to occur in extranodal sites, and clinical and pathological characteristics of extranodal FDC sarcomas remain to be defined. The lack of a high index of suspicion is the main reason for misdiagnosis. The authors point out the difficulties in the diagnosis and management of this rare condition.
Accepted for publication 24 June 2011 Available online 10 August 2011
Follicular dendritic cells (FDCs) are part of the accessory immune system and are normally found in the germinal centres of the secondary lymphoid follicles. Their main functions are the capture and presentation of the antigen and immune complexes, serving as antigen presenting cells and playing a major role in the induction and maintenance of the humoral immune response2,8. According to the World Health Organization (WHO), FDC sarcomas are included in histiocytic and dendritic cell neoplasms2. Many reports emphasize FDC sarcomas arising in lymph nodes1,6,9, and as the morphologic features of FDC sarcomas have become better known, the possibility of this diagnosis is now commonly being considered, especially in nodal-based tumours. FDC sarcoma in extranodal sites seems to be much less recognized, and is generally not considered in the clinical or pathological differential diagnosis of extranodal sites. The authors present a case of FDC
in adenoidectomy and then endoscopically visualized the nasopharynx intraoperatively. There was free margin of tumour. Pathological examination of the specimen revealed proliferation of spindle to ovoid cells with whorled, storiform, and fusiform patterns (Fig. 1). Immunohistochemically, the tumour cells expressed positivity for CD21, CD23, HLA-DR, fascin, and vimentin (Fig. 2); they did not express muscle markers (smooth muscle actin, desmin, and muscle-specific antigen). The results were negative for epithelial markers (pancytokeratin, epithelial membrane antigen) and S-100 protein, as well as antibodies to CD68, CD34, CD20, CD3, and CD1a. Owing to the difficulty of achieving a disease-free space in the nasopharynx, the authors recommended external radiotherapy, but the patient rejected that option. Eight months later, positron emission tomography of the whole body detected no malignancy, locally or distantly.
0901-5027/020218 + 03 $36.00/0
sarcoma and emphasize the difficulties associated with the diagnosis and management of the disease. Case report
A 70-year-old woman was admitted with a nasal obstruction of 5 months’ duration. During clinical examination a mass was discovered in the nasopharynx that was red-brown in colour and approximately 3 cm 3 cm 2 cm in size. There was no ulceration or bleeding focus on it. Computed tomography and magnetic resonance imaging findings disclosed a nasopharyngeal mass without invasion to the clivus or parapharyngeal spaces (Fig. 3). No problems with the cranial nerves were detected in the clinical examination. The patient requested minimally invasive surgery for the nasal obstruction, and rejected postoperative radiotherapy or chemotherapy. The authors performed curettage of the nasopharyngeal mass as
# 2011 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
Follicular dendritic cell
Fig. 1. Neoplastic infiltration with characteristic whorled, dendritic and fusiform pattern (haematoxylin–eosin, 200).
Over the course of 3 years, the patient had two local recurrences and two local surgeries for them. The first recurrence was revealed in the 12-month follow up. Forty-6 months after the initial diagnosis, the patient has disease with advanced locoregional recurrence and multiple focuses with high fluorodeoxyglucose (FDG) positivity in the lungs and iliac bone, which were absent in previous examinations. She refuses any treatment other than the minimal surgery for relieving the nasal obstruction. Discussion
FDC sarcomas primarily affect the young or middle-aged adults. No gender predilection has been reported; however, a slight female predominance (1.2:1) with a median age of 41.5 years has been determined8. The head and neck region is the most common location for extranodal FDC sarcomas,
particularly the tonsils7. FDC sarcoma of the nasopharynx is limited to 7 previously reported cases. According to the WHO classification of tumours of the hematopoietic and lymphoid tissues, these tumours belong to histiocytic and dendritic cell neoplasms2. Extranodal FDC sarcomas, like their nodal counterparts, typically have distinct histological, immunophenotypical, and ultrastructural findings that allow a definitive diagnosis2,8. At least one of the following should be positive for an FDC diagnosis: CD 21, CD 35, CD 23, Ki-M4p, and CAN.422. Clusterin is always strongly positive; in addition, desmoplakin, vimentin, fascin, EGF receptor, and HLA-DR are usually positive for FDC. FDC may show variable staining for epithelial membrane antigen (EMA), S 100 protein, and CD 68. Occasionally cytokeratin, CD 45, and CD 20 display positivity for FDC. But the following are precisely negative for
Fig. 2. Diffuse strong CD 21 immunoreactivity (AEC chromogen, 100).
219
FDC sarcomas: CD 1a, lyzozime, myeloperoxidase, CD 34, CD 33, CD 79 a, CD 30, and HMB 452. The lack of a high index of suspicion is the main reason this tumour is misdiagnosed, because FDC markers are not routinely used in immunohistochemical studies of poorly differentiated tumours. These tumours should be considered as being low- or intermediate-grade malignancy, and the prognosis seems to be good for patients who receive early treatment10. Evolution can be marked by local recurrences and metastases to sites such as the lungs, liver, and lymph nodes4–6. The local recurrence rate is at least 40%, and the metastatic rate is at least 25%3. No deaths have been reported from FDC sarcoma of the nasopharynx8. Owing to the short-term follow up periods in the reported cases, the local recurrence and metastatic rates might be underestimated. Differential diagnosis of FDC includes ectopic meningioma, ectopic or orthotopic thymoma, undifferentiated carcinoma, malignant fibrous histiocytoma, large cell lymphoma, metastatic carcinoma, gastrointestinal tumours, and peripheral nerve sheath tumours5. Some authors have proposed the following criteria for poor prognosis: tumour size, nuclear pleomorphism, mitotic count, intra-abdominal location, lack of adjuvant therapy, coagulative necrosis, and significant cellular atypia1,6. Other than cellular atypia, none of the factors mentioned above showed significant statistical power in predicting tumour recurrence, and it is questionable whether a more poorly differentiated FDC sarcoma will show a more aggressive clinical pattern8. To the best of the authors’ knowledge, there is no reported prospective study of treatments and results in patients with FDC sarcomas, owing to the low number of cases, so it can be considered normal to have controversies regarding treatment. As with any other sarcoma, resection of the tumour with a wide margin is generally recommended whenever feasible. As in this case, surgery alone generally does not provide disease-free survival. The authors want to highlight the importance of adjunctive treatment modalities. FDC sarcomas should be considered as intermediate-grade malignancies. In previous studies, all patients with disease of the nasopharynx had radiotherapy or chemotherapy in addition to surgery: all of the cases had surgery and postoperative radiotherapy except one that had neoadjuvant chemotherapy and surgery. Despite the authors’ urging, the patient in the
220
Karabulut et al.
3.
4.
5.
6.
7.
Fig. 3. T1 weight magnetic resonance imaging shows the mass filling the nasopharynx.
8.
present case is the first to have only one treatment modality. Although there have been no reported deaths from nasopharynx disease in the case studies, surgery does not seem to be enough to treat these patients, possibly due to the location, where it is difficult to achieve a wide free margin of tumour. Tumour behaviour may also be a factor, as FDC sarcomas, specifically extranodal ones, have a high incidence of local and distant metastasis. The number of patients studied is not enough to produce a definitive decision, but it seems that the best course for FDC sarcoma patients with nasopharynx involvement is to have postoperative radiotherapy to prevent locoregional recurrences. If there are any predictors of aggression of the tumour, as previously described, systemic chemotherapy (neo- or adjunctive) can be added to treatment.
Funding
None. Competing interests
None declared.
9.
Ethical approval
Not required. 10.
References 1. Chan JKC, Fletcher CDM, Nayler SJ, Cooper K. Follicular dendritic cell sarcoma clinicopathologic analysis of 17 cases suggesting a malignant potential higher than currently recognized. Cancer 1997;79:294–313. 2. Chan JKC, Pileri SA, Delsol G, Fletcher CDM, Weiss LM, Grogg KL. Follicular dendritic cell sarcoma. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA,
Stein H, Thiele J, Vardiman JW, editors. World Health Organization classification of tumors of haematopoietic and lymphoid tissues. Lyon: IARC Press; 2008. p. 363–4. Choi PC, To KF, Lai FM, Lee TW, Yim AP, Chan JK. Follicular dendritic cell sarcoma of the neck: report of two cases complicated by pulmonary metastases. Cancer 2000; 89:664–72. Desai S, Deshpande RB, Jambhekar N. Follicular dendritic cell tumor of the parapharyngeal region. Head Neck 1999;21:164–7. Domı´nguez-Malago´n H, Cano-Valdez AM, Mosqueda-Taylor A, Hes O. Follicular dendritic cell sarcoma of the pharyngeal region: histologic, cytologic, immunohistochemical, and ultrastructural study of three cases. Ann Diagn Pathol 2004; 8:325–32. Perez-Ordonez B, Erlandson RA, Rosai J. Follicular dendritic cell tumor: report of 13 additional cases of a distinctive entity. Am J Surg Pathol 1996;20:944–55. Satoh K, Hibi G, Yamamoto Y, Urano M, Kuroda M, Nakamura S. Follicular dendritic cell tumor in the oropharyngeal region: report of a case and review of the literature. Oral Oncol 2003;39:415–9. Shia J, Chen W, Tang LH, Carlson DL, Qin J, Guillem JG, Nobrega J, Wong WD, Klimstra DS. Extranodal follicular dendritic cell sarcoma: clinical, pathologic, and histogenetic characteristics of an underrecognized disease entity. Virchows Arch 2006;449:148–58. Soriano AO, Thompson MA, Admirand JH, Fayad LE, Rodriguez AM, Romaguera JE, Hagemeister FB, Pro B. Follicular dendritic cell sarcoma: a report of 14 cases and a review of the literature. Am J Hematol 2007;82:725–8. Tisch M, Hengstermann F, Kraft K, Von Hinu¨ber G, Maier H. Follicular dendritic cell sarcoma of the tonsil: report of a rare case. Ear Nose Throat J 2003;82:507–9.
Address: Burak Karabulut Ardahan Devlet Hastanesi KBB Hastalıkları Birimi Ardahan Turkey Tel.: +90 505 244 97 97/478 2112909 E-mail: [email protected]
Case Report Head and Neck Oncology
Follicular dendritic cell sarcoma of the nasopharynx
B. Karabulut1, K. S. Orhan2, Y. Guldiken2, O. Dogan3 1
Department of Otolaryngology, Ardahan State Hospital, Turkey; 2Department of Otolaryngology Head and Neck Surgery, Istanbul University, Istanbul Medical Faculty, Turkey; 3Department of Pathology, Istanbul University, Istanbul Medical Faculty, Turkey
B. Karabulut, K. S. Orhan, Y. Guldiken, O. Dogan: Follicular dendritic cell sarcoma of the nasopharynx. Int. J. Oral Maxillofac. Surg. 2012; 41: 218–220. # 2011 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved. Abstract. Follicular dendritic cell (FDC) sarcomas of the nasopharynx are rare tumours; only seven cases have been reported in the English language medical literature. The authors present an eighth case, which occurred in a 70-year-old woman whose main complaint was nasal obstruction. It has been more than 10 years since FDC sarcoma was reported to occur in extranodal sites, and clinical and pathological characteristics of extranodal FDC sarcomas remain to be defined. The lack of a high index of suspicion is the main reason for misdiagnosis. The authors point out the difficulties in the diagnosis and management of this rare condition.
Accepted for publication 24 June 2011 Available online 10 August 2011
Follicular dendritic cells (FDCs) are part of the accessory immune system and are normally found in the germinal centres of the secondary lymphoid follicles. Their main functions are the capture and presentation of the antigen and immune complexes, serving as antigen presenting cells and playing a major role in the induction and maintenance of the humoral immune response2,8. According to the World Health Organization (WHO), FDC sarcomas are included in histiocytic and dendritic cell neoplasms2. Many reports emphasize FDC sarcomas arising in lymph nodes1,6,9, and as the morphologic features of FDC sarcomas have become better known, the possibility of this diagnosis is now commonly being considered, especially in nodal-based tumours. FDC sarcoma in extranodal sites seems to be much less recognized, and is generally not considered in the clinical or pathological differential diagnosis of extranodal sites. The authors present a case of FDC
in adenoidectomy and then endoscopically visualized the nasopharynx intraoperatively. There was free margin of tumour. Pathological examination of the specimen revealed proliferation of spindle to ovoid cells with whorled, storiform, and fusiform patterns (Fig. 1). Immunohistochemically, the tumour cells expressed positivity for CD21, CD23, HLA-DR, fascin, and vimentin (Fig. 2); they did not express muscle markers (smooth muscle actin, desmin, and muscle-specific antigen). The results were negative for epithelial markers (pancytokeratin, epithelial membrane antigen) and S-100 protein, as well as antibodies to CD68, CD34, CD20, CD3, and CD1a. Owing to the difficulty of achieving a disease-free space in the nasopharynx, the authors recommended external radiotherapy, but the patient rejected that option. Eight months later, positron emission tomography of the whole body detected no malignancy, locally or distantly.
0901-5027/020218 + 03 $36.00/0
sarcoma and emphasize the difficulties associated with the diagnosis and management of the disease. Case report
A 70-year-old woman was admitted with a nasal obstruction of 5 months’ duration. During clinical examination a mass was discovered in the nasopharynx that was red-brown in colour and approximately 3 cm 3 cm 2 cm in size. There was no ulceration or bleeding focus on it. Computed tomography and magnetic resonance imaging findings disclosed a nasopharyngeal mass without invasion to the clivus or parapharyngeal spaces (Fig. 3). No problems with the cranial nerves were detected in the clinical examination. The patient requested minimally invasive surgery for the nasal obstruction, and rejected postoperative radiotherapy or chemotherapy. The authors performed curettage of the nasopharyngeal mass as
# 2011 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
Follicular dendritic cell
Fig. 1. Neoplastic infiltration with characteristic whorled, dendritic and fusiform pattern (haematoxylin–eosin, 200).
Over the course of 3 years, the patient had two local recurrences and two local surgeries for them. The first recurrence was revealed in the 12-month follow up. Forty-6 months after the initial diagnosis, the patient has disease with advanced locoregional recurrence and multiple focuses with high fluorodeoxyglucose (FDG) positivity in the lungs and iliac bone, which were absent in previous examinations. She refuses any treatment other than the minimal surgery for relieving the nasal obstruction. Discussion
FDC sarcomas primarily affect the young or middle-aged adults. No gender predilection has been reported; however, a slight female predominance (1.2:1) with a median age of 41.5 years has been determined8. The head and neck region is the most common location for extranodal FDC sarcomas,
particularly the tonsils7. FDC sarcoma of the nasopharynx is limited to 7 previously reported cases. According to the WHO classification of tumours of the hematopoietic and lymphoid tissues, these tumours belong to histiocytic and dendritic cell neoplasms2. Extranodal FDC sarcomas, like their nodal counterparts, typically have distinct histological, immunophenotypical, and ultrastructural findings that allow a definitive diagnosis2,8. At least one of the following should be positive for an FDC diagnosis: CD 21, CD 35, CD 23, Ki-M4p, and CAN.422. Clusterin is always strongly positive; in addition, desmoplakin, vimentin, fascin, EGF receptor, and HLA-DR are usually positive for FDC. FDC may show variable staining for epithelial membrane antigen (EMA), S 100 protein, and CD 68. Occasionally cytokeratin, CD 45, and CD 20 display positivity for FDC. But the following are precisely negative for
Fig. 2. Diffuse strong CD 21 immunoreactivity (AEC chromogen, 100).
219
FDC sarcomas: CD 1a, lyzozime, myeloperoxidase, CD 34, CD 33, CD 79 a, CD 30, and HMB 452. The lack of a high index of suspicion is the main reason this tumour is misdiagnosed, because FDC markers are not routinely used in immunohistochemical studies of poorly differentiated tumours. These tumours should be considered as being low- or intermediate-grade malignancy, and the prognosis seems to be good for patients who receive early treatment10. Evolution can be marked by local recurrences and metastases to sites such as the lungs, liver, and lymph nodes4–6. The local recurrence rate is at least 40%, and the metastatic rate is at least 25%3. No deaths have been reported from FDC sarcoma of the nasopharynx8. Owing to the short-term follow up periods in the reported cases, the local recurrence and metastatic rates might be underestimated. Differential diagnosis of FDC includes ectopic meningioma, ectopic or orthotopic thymoma, undifferentiated carcinoma, malignant fibrous histiocytoma, large cell lymphoma, metastatic carcinoma, gastrointestinal tumours, and peripheral nerve sheath tumours5. Some authors have proposed the following criteria for poor prognosis: tumour size, nuclear pleomorphism, mitotic count, intra-abdominal location, lack of adjuvant therapy, coagulative necrosis, and significant cellular atypia1,6. Other than cellular atypia, none of the factors mentioned above showed significant statistical power in predicting tumour recurrence, and it is questionable whether a more poorly differentiated FDC sarcoma will show a more aggressive clinical pattern8. To the best of the authors’ knowledge, there is no reported prospective study of treatments and results in patients with FDC sarcomas, owing to the low number of cases, so it can be considered normal to have controversies regarding treatment. As with any other sarcoma, resection of the tumour with a wide margin is generally recommended whenever feasible. As in this case, surgery alone generally does not provide disease-free survival. The authors want to highlight the importance of adjunctive treatment modalities. FDC sarcomas should be considered as intermediate-grade malignancies. In previous studies, all patients with disease of the nasopharynx had radiotherapy or chemotherapy in addition to surgery: all of the cases had surgery and postoperative radiotherapy except one that had neoadjuvant chemotherapy and surgery. Despite the authors’ urging, the patient in the
220
Karabulut et al.
3.
4.
5.
6.
7.
Fig. 3. T1 weight magnetic resonance imaging shows the mass filling the nasopharynx.
8.
present case is the first to have only one treatment modality. Although there have been no reported deaths from nasopharynx disease in the case studies, surgery does not seem to be enough to treat these patients, possibly due to the location, where it is difficult to achieve a wide free margin of tumour. Tumour behaviour may also be a factor, as FDC sarcomas, specifically extranodal ones, have a high incidence of local and distant metastasis. The number of patients studied is not enough to produce a definitive decision, but it seems that the best course for FDC sarcoma patients with nasopharynx involvement is to have postoperative radiotherapy to prevent locoregional recurrences. If there are any predictors of aggression of the tumour, as previously described, systemic chemotherapy (neo- or adjunctive) can be added to treatment.
Funding
None. Competing interests
None declared.
9.
Ethical approval
Not required. 10.
References 1. Chan JKC, Fletcher CDM, Nayler SJ, Cooper K. Follicular dendritic cell sarcoma clinicopathologic analysis of 17 cases suggesting a malignant potential higher than currently recognized. Cancer 1997;79:294–313. 2. Chan JKC, Pileri SA, Delsol G, Fletcher CDM, Weiss LM, Grogg KL. Follicular dendritic cell sarcoma. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA,
Stein H, Thiele J, Vardiman JW, editors. World Health Organization classification of tumors of haematopoietic and lymphoid tissues. Lyon: IARC Press; 2008. p. 363–4. Choi PC, To KF, Lai FM, Lee TW, Yim AP, Chan JK. Follicular dendritic cell sarcoma of the neck: report of two cases complicated by pulmonary metastases. Cancer 2000; 89:664–72. Desai S, Deshpande RB, Jambhekar N. Follicular dendritic cell tumor of the parapharyngeal region. Head Neck 1999;21:164–7. Domı´nguez-Malago´n H, Cano-Valdez AM, Mosqueda-Taylor A, Hes O. Follicular dendritic cell sarcoma of the pharyngeal region: histologic, cytologic, immunohistochemical, and ultrastructural study of three cases. Ann Diagn Pathol 2004; 8:325–32. Perez-Ordonez B, Erlandson RA, Rosai J. Follicular dendritic cell tumor: report of 13 additional cases of a distinctive entity. Am J Surg Pathol 1996;20:944–55. Satoh K, Hibi G, Yamamoto Y, Urano M, Kuroda M, Nakamura S. Follicular dendritic cell tumor in the oropharyngeal region: report of a case and review of the literature. Oral Oncol 2003;39:415–9. Shia J, Chen W, Tang LH, Carlson DL, Qin J, Guillem JG, Nobrega J, Wong WD, Klimstra DS. Extranodal follicular dendritic cell sarcoma: clinical, pathologic, and histogenetic characteristics of an underrecognized disease entity. Virchows Arch 2006;449:148–58. Soriano AO, Thompson MA, Admirand JH, Fayad LE, Rodriguez AM, Romaguera JE, Hagemeister FB, Pro B. Follicular dendritic cell sarcoma: a report of 14 cases and a review of the literature. Am J Hematol 2007;82:725–8. Tisch M, Hengstermann F, Kraft K, Von Hinu¨ber G, Maier H. Follicular dendritic cell sarcoma of the tonsil: report of a rare case. Ear Nose Throat J 2003;82:507–9.
Address: Burak Karabulut Ardahan Devlet Hastanesi KBB Hastalıkları Birimi Ardahan Turkey Tel.: +90 505 244 97 97/478 2112909 E-mail: [email protected]