An Unusual Pulmonary Mass With Mediastinal Invasion and Multiple Intrapulmonary Nodules in a 52-Year-Old Man
2012; Elsevier BV; Volume: 141; Issue: 1 Linguagem: Inglês
10.1378/chest.11-0360
ISSN1931-3543
AutoresChih‐Jen Yang, Jui‐Ying Lee, Chun‐Chieh Wu, Hsin‐Ling Yin, Chi‐Tun Lien, Yi‐Chang Liu,
Tópico(s)Viral-associated cancers and disorders
ResumoA 52-year-old previously healthy smoker was examined. He had a fever of up to 38°C accompanied by cough with yellowish sputum for about 1 month. He visited our chest clinic for assistance in November 2010. His physical examination revealed clear breathing sounds but a palpable, fixed lymph node approximately 2 cm×1.5 cm in size over the right supraclavicular area. Laboratory data showed leukocytosis (WBC count, 14,600/μL) with neutrophilia (segment form 78%). His carcinoembryonic antigen level was 0.54 ng/mL (normal <5 ng/mL); lactate dehydrogenase level, 142 IU/L (normal 91-180 IU/L); prostate-specific antigen level, 0.28 ng/dL (normal 25 mitoses per 10 high-power fields was seen (Fig 3B). Immunohistochemical stains were positive for S-100 (Fig 3C) and CD1a (Fig 3D), indicating Langerhans cells. In addition, the tumor cells were positive for CD68. Diagnosis: Pulmonary Langerhans cell sarcoma Neoplasms of dendritic or histiocytic cells are extremely rare. Tumors of the dendritic cell linage currently classified by the World Health Organization (WHO) include follicular dendritic cell tumors, interdigitating dendritic cell tumors, Langerhans cell histiocytosis (LCH), and Langerhans cell sarcoma (LCS).1Swerdlow SH Campo E Harris NL et al.WHO Classification of Tumours of Hematopoietic and Lymphoid Tissues. IARC, Lyon, France2008: 358Google Scholar LCS is a neoplastic proliferation of Langerhans cells with overtly malignant cytologic features and is considered a higher-grade variant of LCH.1Swerdlow SH Campo E Harris NL et al.WHO Classification of Tumours of Hematopoietic and Lymphoid Tissues. IARC, Lyon, France2008: 358Google Scholar LCS arising from an antecedent LCH has been previously reported.2Lee JS Ko GH Kim HC Jang IS Jeon KN Lee JH Langerhans cell sarcoma arising from Langerhans cell histiocytosis: a case report.J Korean Med Sci. 2006; 21: 577-580Crossref PubMed Scopus (53) Google Scholar LCS is often shown as a locally aggressive solitary mass with the ability to recur and metastasize. Only 27 patients with LCS (including the present patient) have been documented in the English literature according to a MEDLINE search,2Lee JS Ko GH Kim HC Jang IS Jeon KN Lee JH Langerhans cell sarcoma arising from Langerhans cell histiocytosis: a case report.J Korean Med Sci. 2006; 21: 577-580Crossref PubMed Scopus (53) Google Scholar, 3Bohn OL Ruiz-Argüelles G Navarro L Saldivar J Sanchez-Sosa S Cutaneous Langerhans cell sarcoma: a case report and review of the literature.Int J Hematol. 2007; 85: 116-120Crossref PubMed Scopus (47) Google Scholar, 4Ferringer T Banks PM Metcalf JS Langerhans cell sarcoma.Am J Dermatopathol. 2006; 28: 36-39Crossref PubMed Scopus (52) Google Scholar, 5Jülg BD Weidner S Mayr D Pulmonary manifestation of a Langerhans cell sarcoma: case report and review of the literature.Virchows Arch. 2006; 448: 369-374Crossref PubMed Scopus (35) Google Scholar, 6Lauritzen AF Delsol G Hansen NE et al.Histiocytic sarcomas and monoblastic leukemias. A clinical, histologic, and immunophenotypical study.Am J Clin Pathol. 1994; 102: 45-54Crossref PubMed Scopus (67) Google Scholar, 7Nakayama M Takahashi K Hori M et al.Langerhans cell sarcoma of the cervical lymph node: a case report and literature review.Auris Nasus Larynx. 2010; 37: 750-753Abstract Full Text Full Text PDF PubMed Scopus (30) Google Scholar, 8Pileri SA Grogan TM Harris NL et al.Tumours of histiocytes and accessory dendritic cells: an immunohistochemical approach to classification from the International Lymphoma Study Group based on 61 cases.Histopathology. 2002; 41: 1-29Crossref PubMed Scopus (529) Google Scholar, 9Zhao G Luo M Wu ZY et al.Langerhans cell sarcoma involving gallbladder and peritoneal lymph nodes: a case report.Int J Surg Pathol. 2009; 17: 347-353Crossref PubMed Scopus (26) Google Scholar, 10Itoh H Miyaguni H Kataoka H et al.Primary cutaneous Langerhans cell histiocytosis showing malignant phenotype in an elderly woman: report of a fatal case.J Cutan Pathol. 2001; 28: 371-378Crossref PubMed Scopus (44) Google Scholar, 11Misery L Godard W Hamzeh H et al.Malignant Langerhans cell tumor: a case with a favorable outcome associated with the absence of blood dendritic cell proliferation.J Am Acad Dermatol. 2003; 49: 527-529Abstract Full Text Full Text PDF PubMed Scopus (27) Google Scholar, 12Uchida K Kobayashi S Inukai T et al.Langerhans cell sarcoma emanating from the upper arm skin: successful treatment by MAID regimen.J Orthop Sci. 2008; 13: 89-93Abstract Full Text PDF PubMed Scopus (24) Google Scholar, 13Kawase T Hamazaki M Ogura M et al.CD56/NCAM-positive Langerhans cell sarcoma: a clinicopathologic study of 4 cases.Int J Hematol. 2005; 81: 323-329Crossref PubMed Scopus (54) Google Scholar, 14Stacher E Beham-Schmid C Terpe HJ Simiantonaki N Popper HH Pulmonary histiocytic sarcoma mimicking pulmonary Langerhans cell histiocytosis in a young adult presenting with spontaneous pneumothorax: a potential diagnostic pitfall.Virchows Arch. 2009; 455: 187-190Crossref PubMed Scopus (12) Google Scholar, 15Sumida K Yoshidomi Y Koga H et al.Leukemic transformation of Langerhans cell sarcoma.Int J Hematol. 2008; 87: 527-531Crossref PubMed Scopus (23) Google Scholar, 16Yoshimi A Kumano K Motokura T et al.ESHAP therapy effective in a patient with Langerhans cell sarcoma.Int J Hematol. 2008; 87: 532-537Crossref PubMed Scopus (24) Google Scholar highlighting the rarity of the disease. Among these patients, pulmonary LCS is extremely rare.2Lee JS Ko GH Kim HC Jang IS Jeon KN Lee JH Langerhans cell sarcoma arising from Langerhans cell histiocytosis: a case report.J Korean Med Sci. 2006; 21: 577-580Crossref PubMed Scopus (53) Google Scholar, 5Jülg BD Weidner S Mayr D Pulmonary manifestation of a Langerhans cell sarcoma: case report and review of the literature.Virchows Arch. 2006; 448: 369-374Crossref PubMed Scopus (35) Google Scholar Because of the rarity of the malignancy, we reviewed all patients with Langerhans cell sarcoma in the published English literature and summarized this information in Table 1.17Tani M Ishii N Kumagai M Ban M Sasase A Mishima Y Malignant Langerhans cell tumour.Br J Dermatol. 1992; 126: 398-403Crossref PubMed Scopus (40) Google Scholar From this review, the characteristics of LCS included an age range of 10 to 81 years, a slight male predominance, and that most patients had multiple organ involvement presenting with local or systemic presentations.8Pileri SA Grogan TM Harris NL et al.Tumours of histiocytes and accessory dendritic cells: an immunohistochemical approach to classification from the International Lymphoma Study Group based on 61 cases.Histopathology. 2002; 41: 1-29Crossref PubMed Scopus (529) Google Scholar, 13Kawase T Hamazaki M Ogura M et al.CD56/NCAM-positive Langerhans cell sarcoma: a clinicopathologic study of 4 cases.Int J Hematol. 2005; 81: 323-329Crossref PubMed Scopus (54) Google Scholar, 16Yoshimi A Kumano K Motokura T et al.ESHAP therapy effective in a patient with Langerhans cell sarcoma.Int J Hematol. 2008; 87: 532-537Crossref PubMed Scopus (24) Google Scholar According to the 2008 WHO classification of tumors of hematopoietic and lymphoid tissues,1Swerdlow SH Campo E Harris NL et al.WHO Classification of Tumours of Hematopoietic and Lymphoid Tissues. IARC, Lyon, France2008: 358Google Scholar by the degree of cytologic atypia and clinical aggressiveness, tumors derived from Langerhans cells can be divided into two main subgroups, LCH and LCS. Clinicopathologic studies are necessary to clarify the nature of the tumor. In LCS, the most prominent feature is the overtly malignant cytology of a pleomorphic tumor, and only the phenotype and/or ultrastructure will reveal Langerhans cell derivation. Until now, only one LCS case has been reported to originate from antecedent LCH,2Lee JS Ko GH Kim HC Jang IS Jeon KN Lee JH Langerhans cell sarcoma arising from Langerhans cell histiocytosis: a case report.J Korean Med Sci. 2006; 21: 577-580Crossref PubMed Scopus (53) Google Scholar suggesting that LCS may present as a de novo picture, as in this case.Table 1Summary of All Reported Cases of Langerhans Cell Sarcoma, According to WHO CriteriaStudy/YearCase No.Age, y/SexInitial SitesTherapyOutcomeTani et al17Tani M Ishii N Kumagai M Ban M Sasase A Mishima Y Malignant Langerhans cell tumour.Br J Dermatol. 1992; 126: 398-403Crossref PubMed Scopus (40) Google Scholar/1992149/FLN, skinChemotherapyDied at 2 moLauritzen et al6Lauritzen AF Delsol G Hansen NE et al.Histiocytic sarcomas and monoblastic leukemias. A clinical, histologic, and immunophenotypical study.Am J Clin Pathol. 1994; 102: 45-54Crossref PubMed Scopus (67) Google Scholar/1994238/MSkin, LN, lungChemotherapyPartial remission after 12 moItoh et al10Itoh H Miyaguni H Kataoka H et al.Primary cutaneous Langerhans cell histiocytosis showing malignant phenotype in an elderly woman: report of a fatal case.J Cutan Pathol. 2001; 28: 371-378Crossref PubMed Scopus (44) Google Scholar/2001374/FSkinSurgery, radiotherapyDied at 14 moPileri et al8Pileri SA Grogan TM Harris NL et al.Tumours of histiocytes and accessory dendritic cells: an immunohistochemical approach to classification from the International Lymphoma Study Group based on 61 cases.Histopathology. 2002; 41: 1-29Crossref PubMed Scopus (529) Google Scholar/2002417/FLNChemotherapyAlive with diseasePileri et al8Pileri SA Grogan TM Harris NL et al.Tumours of histiocytes and accessory dendritic cells: an immunohistochemical approach to classification from the International Lymphoma Study Group based on 61 cases.Histopathology. 2002; 41: 1-29Crossref PubMed Scopus (529) Google Scholar/2002546/MLNChemotherapyAlive with diseasePileri et al8Pileri SA Grogan TM Harris NL et al.Tumours of histiocytes and accessory dendritic cells: an immunohistochemical approach to classification from the International Lymphoma Study Group based on 61 cases.Histopathology. 2002; 41: 1-29Crossref PubMed Scopus (529) Google Scholar/2002628/MMediastinum, liver, spleenNoneDied at 3 wkPileri et al8Pileri SA Grogan TM Harris NL et al.Tumours of histiocytes and accessory dendritic cells: an immunohistochemical approach to classification from the International Lymphoma Study Group based on 61 cases.Histopathology. 2002; 41: 1-29Crossref PubMed Scopus (529) Google Scholar/2002750/FSkinNot availableNot availablePileri et al8Pileri SA Grogan TM Harris NL et al.Tumours of histiocytes and accessory dendritic cells: an immunohistochemical approach to classification from the International Lymphoma Study Group based on 61 cases.Histopathology. 2002; 41: 1-29Crossref PubMed Scopus (529) Google Scholar/2002810/FSkinSurgery, radiotherapyAlive with diseasePileri et al8Pileri SA Grogan TM Harris NL et al.Tumours of histiocytes and accessory dendritic cells: an immunohistochemical approach to classification from the International Lymphoma Study Group based on 61 cases.Histopathology. 2002; 41: 1-29Crossref PubMed Scopus (529) Google Scholar/2002923/FLN, skin, lungChemotherapyDied at 2 yPileri et al8Pileri SA Grogan TM Harris NL et al.Tumours of histiocytes and accessory dendritic cells: an immunohistochemical approach to classification from the International Lymphoma Study Group based on 61 cases.Histopathology. 2002; 41: 1-29Crossref PubMed Scopus (529) Google Scholar/20021065/FLN, liver, spleen, lungChemotherapyDied of diseasePileri et al8Pileri SA Grogan TM Harris NL et al.Tumours of histiocytes and accessory dendritic cells: an immunohistochemical approach to classification from the International Lymphoma Study Group based on 61 cases.Histopathology. 2002; 41: 1-29Crossref PubMed Scopus (529) Google Scholar/20021172/MLN, lung, ribChemotherapyDied of diseasePileri et al8Pileri SA Grogan TM Harris NL et al.Tumours of histiocytes and accessory dendritic cells: an immunohistochemical approach to classification from the International Lymphoma Study Group based on 61 cases.Histopathology. 2002; 41: 1-29Crossref PubMed Scopus (529) Google Scholar/20021250/FBoneSurgeryAlive with diseaseMisery et al11Misery L Godard W Hamzeh H et al.Malignant Langerhans cell tumor: a case with a favorable outcome associated with the absence of blood dendritic cell proliferation.J Am Acad Dermatol. 2003; 49: 527-529Abstract Full Text Full Text PDF PubMed Scopus (27) Google Scholar/20031338/FSkinSurgeryAlive with disease, partial response at 12 moKawase et al13Kawase T Hamazaki M Ogura M et al.CD56/NCAM-positive Langerhans cell sarcoma: a clinicopathologic study of 4 cases.Int J Hematol. 2005; 81: 323-329Crossref PubMed Scopus (54) Google Scholar/20051459/MSpleen, skin, LN, bone marrowChemotherapyDied at 9 yearsKawase et al13Kawase T Hamazaki M Ogura M et al.CD56/NCAM-positive Langerhans cell sarcoma: a clinicopathologic study of 4 cases.Int J Hematol. 2005; 81: 323-329Crossref PubMed Scopus (54) Google Scholar/20051535/MBone, LN, liver, lungChemotherapyDied of diseaseKawase et al13Kawase T Hamazaki M Ogura M et al.CD56/NCAM-positive Langerhans cell sarcoma: a clinicopathologic study of 4 cases.Int J Hematol. 2005; 81: 323-329Crossref PubMed Scopus (54) Google Scholar/20051661/FLN, liver, spleenChemotherapyDied at 10 moKawase et al13Kawase T Hamazaki M Ogura M et al.CD56/NCAM-positive Langerhans cell sarcoma: a clinicopathologic study of 4 cases.Int J Hematol. 2005; 81: 323-329Crossref PubMed Scopus (54) Google Scholar/20051760/MBoneRadiotherapyAliveFerringer et al4Ferringer T Banks PM Metcalf JS Langerhans cell sarcoma.Am J Dermatopathol. 2006; 28: 36-39Crossref PubMed Scopus (52) Google Scholar/20061833/MLN, skinChemotherapyAliveJülg et al5Jülg BD Weidner S Mayr D Pulmonary manifestation of a Langerhans cell sarcoma: case report and review of the literature.Virchows Arch. 2006; 448: 369-374Crossref PubMed Scopus (35) Google Scholar/20061981/MMediastinal mass, lungChemotherapyDied at 4 moLee et al2Lee JS Ko GH Kim HC Jang IS Jeon KN Lee JH Langerhans cell sarcoma arising from Langerhans cell histiocytosis: a case report.J Korean Med Sci. 2006; 21: 577-580Crossref PubMed Scopus (53) Google Scholar/20062034/MLungSurgeryAliveBohn et al3Bohn OL Ruiz-Argüelles G Navarro L Saldivar J Sanchez-Sosa S Cutaneous Langerhans cell sarcoma: a case report and review of the literature.Int J Hematol. 2007; 85: 116-120Crossref PubMed Scopus (47) Google Scholar/20072147/MSkin, LNChemotherapyAliveSumida et al15Sumida K Yoshidomi Y Koga H et al.Leukemic transformation of Langerhans cell sarcoma.Int J Hematol. 2008; 87: 527-531Crossref PubMed Scopus (23) Google Scholar/20082257/MLNChemotherapyDied at 7 moUchida et al12Uchida K Kobayashi S Inukai T et al.Langerhans cell sarcoma emanating from the upper arm skin: successful treatment by MAID regimen.J Orthop Sci. 2008; 13: 89-93Abstract Full Text PDF PubMed Scopus (24) Google Scholar/20082372/MSkinChemotherapy and surgery.AliveZhao et al9Zhao G Luo M Wu ZY et al.Langerhans cell sarcoma involving gallbladder and peritoneal lymph nodes: a case report.Int J Surg Pathol. 2009; 17: 347-353Crossref PubMed Scopus (26) Google Scholar/20092474/FGall bladder, LNSurgery and chemotherapyAliveYoshimi et al16Yoshimi A Kumano K Motokura T et al.ESHAP therapy effective in a patient with Langerhans cell sarcoma.Int J Hematol. 2008; 87: 532-537Crossref PubMed Scopus (24) Google Scholar/200825Skin, LN, lungChemotherapyDied at 5 moNakayama et al7Nakayama M Takahashi K Hori M et al.Langerhans cell sarcoma of the cervical lymph node: a case report and literature review.Auris Nasus Larynx. 2010; 37: 750-753Abstract Full Text Full Text PDF PubMed Scopus (30) Google Scholar/20102662/MLNRadiotherapy, chemotherapyAlivePresent case2752/MLung, LN, ribChemotherapyRefractory to chemotherapyF = female; LN = lymph node; M = male; WHO = World Health Organization. Open table in a new tab F = female; LN = lymph node; M = male; WHO = World Health Organization. Pulmonary LCH is almost always associated with smokers and presents as a rather benign picture. However, LCS is an overtly malignant aggressive tumor, and only rare cases have been reported. The process causing LCS is currently unknown. In addition, only a few reported cases involve patients with a history of smoking, suggesting that in LCS, the association with smoking is unclear and different from LCH. Pulmonary manifestations often present with cough,2Lee JS Ko GH Kim HC Jang IS Jeon KN Lee JH Langerhans cell sarcoma arising from Langerhans cell histiocytosis: a case report.J Korean Med Sci. 2006; 21: 577-580Crossref PubMed Scopus (53) Google Scholar, 5Jülg BD Weidner S Mayr D Pulmonary manifestation of a Langerhans cell sarcoma: case report and review of the literature.Virchows Arch. 2006; 448: 369-374Crossref PubMed Scopus (35) Google Scholar fever,13Kawase T Hamazaki M Ogura M et al.CD56/NCAM-positive Langerhans cell sarcoma: a clinicopathologic study of 4 cases.Int J Hematol. 2005; 81: 323-329Crossref PubMed Scopus (54) Google Scholar dyspnea,14Stacher E Beham-Schmid C Terpe HJ Simiantonaki N Popper HH Pulmonary histiocytic sarcoma mimicking pulmonary Langerhans cell histiocytosis in a young adult presenting with spontaneous pneumothorax: a potential diagnostic pitfall.Virchows Arch. 2009; 455: 187-190Crossref PubMed Scopus (12) Google Scholar respiratory failure,16Yoshimi A Kumano K Motokura T et al.ESHAP therapy effective in a patient with Langerhans cell sarcoma.Int J Hematol. 2008; 87: 532-537Crossref PubMed Scopus (24) Google Scholar and body weight loss.5Jülg BD Weidner S Mayr D Pulmonary manifestation of a Langerhans cell sarcoma: case report and review of the literature.Virchows Arch. 2006; 448: 369-374Crossref PubMed Scopus (35) Google Scholar The diagnosis often relies on bronchoscopic biopsy5Jülg BD Weidner S Mayr D Pulmonary manifestation of a Langerhans cell sarcoma: case report and review of the literature.Virchows Arch. 2006; 448: 369-374Crossref PubMed Scopus (35) Google Scholar or CT scan-guided biopsy.2Lee JS Ko GH Kim HC Jang IS Jeon KN Lee JH Langerhans cell sarcoma arising from Langerhans cell histiocytosis: a case report.J Korean Med Sci. 2006; 21: 577-580Crossref PubMed Scopus (53) Google Scholar However, it is often hard to make a definite diagnosis from the tiny specimens obtained from biopsy, and lobectomy via surgery, such as video-assisted thoracoscopic surgery,2Lee JS Ko GH Kim HC Jang IS Jeon KN Lee JH Langerhans cell sarcoma arising from Langerhans cell histiocytosis: a case report.J Korean Med Sci. 2006; 21: 577-580Crossref PubMed Scopus (53) Google Scholar, 14Stacher E Beham-Schmid C Terpe HJ Simiantonaki N Popper HH Pulmonary histiocytic sarcoma mimicking pulmonary Langerhans cell histiocytosis in a young adult presenting with spontaneous pneumothorax: a potential diagnostic pitfall.Virchows Arch. 2009; 455: 187-190Crossref PubMed Scopus (12) Google Scholar is often necessary, as with this patient. The choices of treatment of LCS include surgical intervention, chemotherapy, radiotherapy, or a combination of the above. Surgery for merely local involvement can lead to long-term survival.2Lee JS Ko GH Kim HC Jang IS Jeon KN Lee JH Langerhans cell sarcoma arising from Langerhans cell histiocytosis: a case report.J Korean Med Sci. 2006; 21: 577-580Crossref PubMed Scopus (53) Google Scholar, 8Pileri SA Grogan TM Harris NL et al.Tumours of histiocytes and accessory dendritic cells: an immunohistochemical approach to classification from the International Lymphoma Study Group based on 61 cases.Histopathology. 2002; 41: 1-29Crossref PubMed Scopus (529) Google Scholar, 9Zhao G Luo M Wu ZY et al.Langerhans cell sarcoma involving gallbladder and peritoneal lymph nodes: a case report.Int J Surg Pathol. 2009; 17: 347-353Crossref PubMed Scopus (26) Google Scholar, 11Misery L Godard W Hamzeh H et al.Malignant Langerhans cell tumor: a case with a favorable outcome associated with the absence of blood dendritic cell proliferation.J Am Acad Dermatol. 2003; 49: 527-529Abstract Full Text Full Text PDF PubMed Scopus (27) Google Scholar, 12Uchida K Kobayashi S Inukai T et al.Langerhans cell sarcoma emanating from the upper arm skin: successful treatment by MAID regimen.J Orthop Sci. 2008; 13: 89-93Abstract Full Text PDF PubMed Scopus (24) Google Scholar However, systemic chemotherapy is often chosen because most patients have multiple organ involvement and distal metastasis. More than one-half of the patients die within 2 years; however, some patients achieve complete remission or remain alive with the disease undergoing surgical intervention and/or radiotherapy.5Jülg BD Weidner S Mayr D Pulmonary manifestation of a Langerhans cell sarcoma: case report and review of the literature.Virchows Arch. 2006; 448: 369-374Crossref PubMed Scopus (35) Google Scholar, 6Lauritzen AF Delsol G Hansen NE et al.Histiocytic sarcomas and monoblastic leukemias. A clinical, histologic, and immunophenotypical study.Am J Clin Pathol. 1994; 102: 45-54Crossref PubMed Scopus (67) Google Scholar, 8Pileri SA Grogan TM Harris NL et al.Tumours of histiocytes and accessory dendritic cells: an immunohistochemical approach to classification from the International Lymphoma Study Group based on 61 cases.Histopathology. 2002; 41: 1-29Crossref PubMed Scopus (529) Google Scholar, 10Itoh H Miyaguni H Kataoka H et al.Primary cutaneous Langerhans cell histiocytosis showing malignant phenotype in an elderly woman: report of a fatal case.J Cutan Pathol. 2001; 28: 371-378Crossref PubMed Scopus (44) Google Scholar, 13Kawase T Hamazaki M Ogura M et al.CD56/NCAM-positive Langerhans cell sarcoma: a clinicopathologic study of 4 cases.Int J Hematol. 2005; 81: 323-329Crossref PubMed Scopus (54) Google Scholar, 15Sumida K Yoshidomi Y Koga H et al.Leukemic transformation of Langerhans cell sarcoma.Int J Hematol. 2008; 87: 527-531Crossref PubMed Scopus (23) Google Scholar, 16Yoshimi A Kumano K Motokura T et al.ESHAP therapy effective in a patient with Langerhans cell sarcoma.Int J Hematol. 2008; 87: 532-537Crossref PubMed Scopus (24) Google Scholar, 17Tani M Ishii N Kumagai M Ban M Sasase A Mishima Y Malignant Langerhans cell tumour.Br J Dermatol. 1992; 126: 398-403Crossref PubMed Scopus (40) Google Scholar Unlike the common chemotherapy prescribed for non-small cell lung cancer, the most common chemotherapy regimens for Langerhans cell sarcoma are lymphomalike regimens, such as CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisolone).5Jülg BD Weidner S Mayr D Pulmonary manifestation of a Langerhans cell sarcoma: case report and review of the literature.Virchows Arch. 2006; 448: 369-374Crossref PubMed Scopus (35) Google Scholar, 12Uchida K Kobayashi S Inukai T et al.Langerhans cell sarcoma emanating from the upper arm skin: successful treatment by MAID regimen.J Orthop Sci. 2008; 13: 89-93Abstract Full Text PDF PubMed Scopus (24) Google Scholar, 16Yoshimi A Kumano K Motokura T et al.ESHAP therapy effective in a patient with Langerhans cell sarcoma.Int J Hematol. 2008; 87: 532-537Crossref PubMed Scopus (24) Google Scholar, 17Tani M Ishii N Kumagai M Ban M Sasase A Mishima Y Malignant Langerhans cell tumour.Br J Dermatol. 1992; 126: 398-403Crossref PubMed Scopus (40) Google Scholar After the definite diagnosis was made in this case, we also immediately began systemic chemotherapy with CHOP (cyclophosphamide 750 mg/m2, doxorubicin 50 mg/m2, vincristine 1.4 mg/m2, and prednisolone 40 mg/m2); however, he proved to be refractory to the regimen after three courses. Uchida et al12Uchida K Kobayashi S Inukai T et al.Langerhans cell sarcoma emanating from the upper arm skin: successful treatment by MAID regimen.J Orthop Sci. 2008; 13: 89-93Abstract Full Text PDF PubMed Scopus (24) Google Scholar followed the treatment protocol for soft tissue sarcoma and used a combination of mesna (2,500 mg/m2), doxorubicin (20 mg/m2), ifosfamide (2,500 mg/m2), and dacarbazine (300 mg/m2) (the MAID regimen) for 3 days as neoadjuvant chemotherapy before surgical excision. The response was unexpectedly good, and the patient underwent successful surgical resection resulting in long-term survival. However, chemotherapy with MAID for LCS is still controversial.18Ohara G Funayama Y Satoh H Uchida K Chemotherapy for Langerhans cell sarcoma.J Orthop Sci. 2009; 14: 242-243Abstract Full Text PDF PubMed Scopus (0) Google Scholar Yoshimi et al16Yoshimi A Kumano K Motokura T et al.ESHAP therapy effective in a patient with Langerhans cell sarcoma.Int J Hematol. 2008; 87: 532-537Crossref PubMed Scopus (24) Google Scholar proposed a modified ESHAP (etoposide, methylprednisolone, cytarabine, and carboplatin) regimen with carboplatin area under the curve 5, mg dose = 5 × (25 + creatinine clearance) continuous IV infusion over 96 h on days 1 to 4, etoposide 60 mg/m2/d IV infusion on days 1 to 4, cytarabine 2,000 mg/m2/d IV infusion over 3 h on day 5, and methylprednisolone 500 mg/d IV infusion on days 1 to 5 as salvage treatment after the initial CHOP regimen failed. This regimen obtained transient improvement on clinical presentations and image findings, although the patient died 3 months after the last course of chemotherapy.16Yoshimi A Kumano K Motokura T et al.ESHAP therapy effective in a patient with Langerhans cell sarcoma.Int J Hematol. 2008; 87: 532-537Crossref PubMed Scopus (24) Google Scholar LCS often shows multiorgan involvement, including the lymph nodes, liver, spleen, lung, and bone.8Pileri SA Grogan TM Harris NL et al.Tumours of histiocytes and accessory dendritic cells: an immunohistochemical approach to classification from the International Lymphoma Study Group based on 61 cases.Histopathology. 2002; 41: 1-29Crossref PubMed Scopus (529) Google Scholar, 13Kawase T Hamazaki M Ogura M et al.CD56/NCAM-positive Langerhans cell sarcoma: a clinicopathologic study of 4 cases.Int J Hematol. 2005; 81: 323-329Crossref PubMed Scopus (54) Google Scholar, 16Yoshimi A Kumano K Motokura T et al.ESHAP therapy effective in a patient with Langerhans cell sarcoma.Int J Hematol. 2008; 87: 532-537Crossref PubMed Scopus (24) Google Scholar Only pulmonary involvement of LCS is rare, and may present with a poorly circumscribed mass with a few small satellite nodules around the main mass2Lee JS Ko GH Kim HC Jang IS Jeon KN Lee JH Langerhans cell sarcoma arising from Langerhans cell histiocytosis: a case report.J Korean Med Sci. 2006; 21: 577-580Crossref PubMed Scopus (53) Google Scholar or multiple tiny nodules with cavities over the lung fields.16Yoshimi A Kumano K Motokura T et al.ESHAP therapy effective in a patient with Langerhans cell sarcoma.Int J Hematol. 2008; 87: 532-537Crossref PubMed Scopus (24) Google Scholar It can present as only pulmonary infiltration or with both lung and mediastinal mass lesions.5Jülg BD Weidner S Mayr D Pulmonary manifestation of a Langerhans cell sarcoma: case report and review of the literature.Virchows Arch. 2006; 448: 369-374Crossref PubMed Scopus (35) Google Scholar, 13Kawase T Hamazaki M Ogura M et al.CD56/NCAM-positive Langerhans cell sarcoma: a clinicopathologic study of 4 cases.Int J Hematol. 2005; 81: 323-329Crossref PubMed Scopus (54) Google Scholar Pleural effusion5Jülg BD Weidner S Mayr D Pulmonary manifestation of a Langerhans cell sarcoma: case report and review of the literature.Virchows Arch. 2006; 448: 369-374Crossref PubMed Scopus (35) Google Scholar, 13Kawase T Hamazaki M Ogura M et al.CD56/NCAM-positive Langerhans cell sarcoma: a clinicopathologic study of 4 cases.Int J Hematol. 2005; 81: 323-329Crossref PubMed Scopus (54) Google Scholar or pneumothorax14Stacher E Beham-Schmid C Terpe HJ Simiantonaki N Popper HH Pulmonary histiocytic sarcoma mimicking pulmonary Langerhans cell histiocytosis in a young adult presenting with spontaneous pneumothorax: a potential diagnostic pitfall.Virchows Arch. 2009; 455: 187-190Crossref PubMed Scopus (12) Google Scholar as the initial presentations of LCS has also been reported. In addition, as with lung cancer, LCS often has a high maximum standardized uptake value in PET scans, and PET scan is also used to monitor and follow up the chemotherapy response of LCS.16Yoshimi A Kumano K Motokura T et al.ESHAP therapy effective in a patient with Langerhans cell sarcoma.Int J Hematol. 2008; 87: 532-537Crossref PubMed Scopus (24) Google Scholar An LCS diagnosis must include the confirmation of the tumor cells as Langerhans cells, and the differential diagnosis should include malignant melanoma, anaplastic carcinoma, diffuse large B-cell lymphoma, anaplastic large cell lymphoma, T-cell lymphomas, and dendritic cell tumors.3Bohn OL Ruiz-Argüelles G Navarro L Saldivar J Sanchez-Sosa S Cutaneous Langerhans cell sarcoma: a case report and review of the literature.Int J Hematol. 2007; 85: 116-120Crossref PubMed Scopus (47) Google Scholar To distinguish the differential diagnosis, a combination of morphologic and immunophenotypic features characterized by immunohistochemical staining is necessary.3Bohn OL Ruiz-Argüelles G Navarro L Saldivar J Sanchez-Sosa S Cutaneous Langerhans cell sarcoma: a case report and review of the literature.Int J Hematol. 2007; 85: 116-120Crossref PubMed Scopus (47) Google Scholar, 19Ben-Ezra J Bailey A Azumi N et al.Malignant histiocytosis X. A distinct clinicopathologic entity.Cancer. 1991; 68: 1050-1060Crossref PubMed Scopus (71) Google Scholar According to the morphology, the malignant cytologic features and very high proliferation rate in Langerhans cell histiocytosis suggest LCS.17Tani M Ishii N Kumagai M Ban M Sasase A Mishima Y Malignant Langerhans cell tumour.Br J Dermatol. 1992; 126: 398-403Crossref PubMed Scopus (40) Google Scholar, 19Ben-Ezra J Bailey A Azumi N et al.Malignant histiocytosis X. A distinct clinicopathologic entity.Cancer. 1991; 68: 1050-1060Crossref PubMed Scopus (71) Google Scholar In immunohistochemical staining, Langerhans cells are always positive for CD1a and S-100 protein, although expression of some histiocytic markers is also common, such as CD68 and some focal or weak expression of lysozyme.3Bohn OL Ruiz-Argüelles G Navarro L Saldivar J Sanchez-Sosa S Cutaneous Langerhans cell sarcoma: a case report and review of the literature.Int J Hematol. 2007; 85: 116-120Crossref PubMed Scopus (47) Google Scholar Of the reported cases in Table 1, almost all patients were shown to be positive for both S-100 and CD1a, and about one-half were positive for CD68 (data not shown). The demonstration of Birbeck granules in neoplastic cells by electron microscopy has also been suggested when making a diagnosis of LCS.19Ben-Ezra J Bailey A Azumi N et al.Malignant histiocytosis X. A distinct clinicopathologic entity.Cancer. 1991; 68: 1050-1060Crossref PubMed Scopus (71) Google Scholar Tani et al17Tani M Ishii N Kumagai M Ban M Sasase A Mishima Y Malignant Langerhans cell tumour.Br J Dermatol. 1992; 126: 398-403Crossref PubMed Scopus (40) Google Scholar summarized the following criteria for the diagnosis of malignant neoplasms of Langerhans cells: (1) proliferation of typical Birbeck granule-containing tumor cells, and (2) malignant cytologic features such as atypia and frequent mitotic figures. In fact, Birbeck granules were found in this patient via electron microscopy. However, immunohistochemical staining is more reliable and widely available compared with electron microscopy, and identification of Birbeck granules is complicated by technical difficulties and artifacts.5Jülg BD Weidner S Mayr D Pulmonary manifestation of a Langerhans cell sarcoma: case report and review of the literature.Virchows Arch. 2006; 448: 369-374Crossref PubMed Scopus (35) Google Scholar, 8Pileri SA Grogan TM Harris NL et al.Tumours of histiocytes and accessory dendritic cells: an immunohistochemical approach to classification from the International Lymphoma Study Group based on 61 cases.Histopathology. 2002; 41: 1-29Crossref PubMed Scopus (529) Google Scholar, 19Ben-Ezra J Bailey A Azumi N et al.Malignant histiocytosis X. A distinct clinicopathologic entity.Cancer. 1991; 68: 1050-1060Crossref PubMed Scopus (71) Google Scholar We present an extremely rare case of pulmonary Langerhans cell sarcoma in a patient who presented with a right upper lung mass including multiple intrapulmonary metastases and mediastinal lymph node involvement. The immunohistochemical staining was positive for CD1a, S-100 protein, and CD68, accompanied by a high mitotic rate indicating a diagnosis of LCS. A CHOP chemotherapy regimen was prescribed; however, a poor response was noted after three courses had been administered.
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