LETTERS TO THE EDITOR
The Fine-Needle Aspiration Cytology of Metastatic Pleomorphic Dermal Sarcoma Dear Dr. Bedrossian: Undifferentiated high-grade sarcomas of the skin, which have until recently been referred to as atypical fibroxanthoma (AFX), are tumors with a characteristic clinical presentation of a superficial, ulcerating mass, usually of the head and neck region of older males, that is limited to the dermis. The recognition that lesions with many of the clinical and pathologic characteristics of AFX can also extend beyond the dermis, with variable necrosis and vascular invasion, and have a more aggressive clinical course, has resulted in a new clinicopathologic entity referred to as pleomorphic dermal sarcoma (PDS).1–3 PDS appears to occupy the middle of a spectrum of aggressive behavior between AFX and the much deeper situated undifferentiated pleomorphic sarcoma (UPS). Given the new and evolving nature of the literature regarding PDS, it is not surprising that that there are no reports of the fine-needle aspiration (FNA) cytology of PDS. We believe this is the first report of the cytologic presentation of a metastatic PDS and its histologic correlate from an 87-year-old male who presented with a rapidly enlarging ear lesion that had the typical clinical and pathologic features of an AFX with multiple recurrences and eventual regional lymph node metastases evaluated by FNA cytology. Diff-Quik and Papanicolaou stain smears showed individually scattered and loosely clustered, markedly atypical cells including multinucleated tumor giant cells, and osteoclastic cells (Figs. 1 and 2). The cytoplasm was abundant and amphophilic with occasional
*Correspondence to: Jan F. Silverman, M.D., Department of Pathology and Laboratory Medicine, Allegheny General Hospital, Pittsburgh, PA. E-mail: [email protected] Received 12 November 2013; revised 24 January 2014; Accepted 24 February 2014 DOI: 10.1002/dc.23145 Published online 19 March 2014 in Wiley Online Library (wileyonlinelibrary.com).
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cells exhibiting a delicate foamy “xanthomatous” appearance. The nuclei were pleomorphic with irregular contours, coarse chromatin, and readily identifiable single and multiple nucleoli. The previously excised ear lesion demonstrated AFX-like features with invasion beyond the dermis into the subcutaneous tissues adjacent to the auricular cartilage. The cells in the excised ear lesion had similar features to the cytologic smears (Fig. 3). From a cytologic perspective, the cells of PDS present with a spectrum of morphologic findings ranging from relatively small, fusiform to spindle-shaped cells having oval nuclei to markedly enlarged, pleomorphic cells with single and multiple irregular nuclei and prominent nucleoli. The cytoplasm is typically abundant, amphophilic and sometimes delicately foamy, and xanthomatous. These cytologic features raise a differential diagnosis that is primarily centered upon PDS/AFX, UPS, sarcomatoid carcinomas, sarcomatoid melanoma, and metastasizing
Fig. 1. Diff-Quik-stained smear of FNA of lymph node metastasis exhibits markedly atypical and variably multinucleated polygonal and spindled cells (3400). [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.] C 2014 WILEY PERIODICALS, INC. V
Diagnostic Cytopathology DOI 10.1002/dc
FINE-NEEDLE ASPIRATION CYTOLOGY OF METASTATIC PDS
Fig. 2. Papanicolaou-stained smear of FNA of lymph node metastasis exhibits markedly atypical and variably multinucleated polygonal and spindled cells (3400). [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]
abnormal mitoses. PDS/AFX is separated from UPS by the relatively rapid clinical presentation and the superficial, cutaneous origin of PDS/AFX in contrast to the deep-seated primary origin of UPS. Review of the literature reveals a few reports of AFXlike lesions that penetrate into the superficial subcutaneous component with frequent recurrences and widely disseminated metastases.6–12 It is these reports that prompted the development of the new clinicopathologic entity of PDS. In the FNA workup of a metastatic high-grade sarcoma, recognition of the above cytologic features and their association with the recently described PDS will prevent an unnecessary and potentially invasive search for more deeply seated sarcomas such as UPS, particularly in patients with a previous diagnosis of AFX. Allan R. Smith, M.D. Oleksandr Yergiyev, M.D. Jan F. Silverman, M.D. Department of Pathology and Laboratory Medicine Allegheny General Hospital Pittsburgh, Pennsylvania
References 1. McCalmont TH. AFX: What we now know. J Cutan Pathol 2011; 38:853–856. 2. McCalmont TH. Correction and clarification regarding AFX and pleomorphic dermal sarcoma. J Cutan Pathol 2012;39:8. 3. Miller K, Goodlad JR, Brenn T. Pleomorphic dermal sarcoma: Adverse histologic features predict aggressive behavior and allow distinction from atypical fibroxanthoma. Am J Surg Pathol 2012;36: 1317–1326. 4. Davidson JS, Demsey D. Atypical fibroxanthoma: Clinicopathologic determinants for recurrence and implications for surgical management. J Surg Oncol 2012;105:559–562. Fig. 3. High-power image of the excised ear lesion demonstrates numerous bizarre multinucleated malignant cells (Hematoxylin and Eosin, 3400). [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]
“benign” cutaneous histiocytoma (BCH).4,5 Immunohistochemical (IHC) studies for epithelial, stromal, and melanocytic lineages should readily identify the mesenchymal origin of PDS, BCH, and UPS and distinguish these from sarcomatoid carcinoma and melanoma.6,7 Unfortunately, there is no consistent IHC profile that is specific for PDS/ AFX, UPS, and BCH. Morphologically, BCH is composed of relatively uniform bland spindle cells with scattered aggregates of cells forming small fascicles, with rare to occasional mitotic figures. In contrast, the cells in PDS/AFX and UPS exhibit significant cytologic atypia with markedly pleomorphic cells containing profoundly atypical nuclei, prominent nucleoli, and numerous
5. Doyle LA, Fletcher CD. Metastasizing “benign” cutaneous fibrous histiocytoma: A clinicopathologic analysis of 16 cases. Am J Surg Pathol 2013;37:484–495. 6. Beer TW, Drury P, Heenan PJ. Atypical fibroxanthoma: A histological and immunohistochemical review of 171 cases. Am J Dermatopathol 2010;32:533–540. 7. Luzar B, Calonje E. Morphological and immunohistochemical characteristics of atypical fibroxanthoma with a special emphasis on potential diagnostic pitfalls: A review. J Cutan Pathol 2010;37:301– 309. 8. Fretzin DF, Helwig EB. Atypical fibroxanthoma of the skin. A clinicopathologic study of 140 cases. Cancer 1973;31:1541–1552. 9. Mirza B, Weedon D. Atypical fibroxanthoma: A clinicopathological study of 89 cases. Australas J Dermatol 2005;46:235–238. 10. Dahl I. Atypical fibroxanthoma of the skin. A clinicopathological study of 57 cases. Acta Pathol Microbiol Scand A 1976;84:183– 197. 11. Helwig EB, May D. Atypical fibroxanthoma of the skin with metastasis. Cancer 1986;57:368–376. 12. Jacobs DS, Edwards WD, Ye RC. Metastatic atypical fibroxanthoma of skin. Cancer 1975;35:457–463.
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The Fine-Needle Aspiration Cytology of Metastatic Pleomorphic Dermal Sarcoma Dear Dr. Bedrossian: Undifferentiated high-grade sarcomas of the skin, which have until recently been referred to as atypical fibroxanthoma (AFX), are tumors with a characteristic clinical presentation of a superficial, ulcerating mass, usually of the head and neck region of older males, that is limited to the dermis. The recognition that lesions with many of the clinical and pathologic characteristics of AFX can also extend beyond the dermis, with variable necrosis and vascular invasion, and have a more aggressive clinical course, has resulted in a new clinicopathologic entity referred to as pleomorphic dermal sarcoma (PDS).1–3 PDS appears to occupy the middle of a spectrum of aggressive behavior between AFX and the much deeper situated undifferentiated pleomorphic sarcoma (UPS). Given the new and evolving nature of the literature regarding PDS, it is not surprising that that there are no reports of the fine-needle aspiration (FNA) cytology of PDS. We believe this is the first report of the cytologic presentation of a metastatic PDS and its histologic correlate from an 87-year-old male who presented with a rapidly enlarging ear lesion that had the typical clinical and pathologic features of an AFX with multiple recurrences and eventual regional lymph node metastases evaluated by FNA cytology. Diff-Quik and Papanicolaou stain smears showed individually scattered and loosely clustered, markedly atypical cells including multinucleated tumor giant cells, and osteoclastic cells (Figs. 1 and 2). The cytoplasm was abundant and amphophilic with occasional
*Correspondence to: Jan F. Silverman, M.D., Department of Pathology and Laboratory Medicine, Allegheny General Hospital, Pittsburgh, PA. E-mail: [email protected] Received 12 November 2013; revised 24 January 2014; Accepted 24 February 2014 DOI: 10.1002/dc.23145 Published online 19 March 2014 in Wiley Online Library (wileyonlinelibrary.com).
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Diagnostic Cytopathology, Vol. 43, No 1
cells exhibiting a delicate foamy “xanthomatous” appearance. The nuclei were pleomorphic with irregular contours, coarse chromatin, and readily identifiable single and multiple nucleoli. The previously excised ear lesion demonstrated AFX-like features with invasion beyond the dermis into the subcutaneous tissues adjacent to the auricular cartilage. The cells in the excised ear lesion had similar features to the cytologic smears (Fig. 3). From a cytologic perspective, the cells of PDS present with a spectrum of morphologic findings ranging from relatively small, fusiform to spindle-shaped cells having oval nuclei to markedly enlarged, pleomorphic cells with single and multiple irregular nuclei and prominent nucleoli. The cytoplasm is typically abundant, amphophilic and sometimes delicately foamy, and xanthomatous. These cytologic features raise a differential diagnosis that is primarily centered upon PDS/AFX, UPS, sarcomatoid carcinomas, sarcomatoid melanoma, and metastasizing
Fig. 1. Diff-Quik-stained smear of FNA of lymph node metastasis exhibits markedly atypical and variably multinucleated polygonal and spindled cells (3400). [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.] C 2014 WILEY PERIODICALS, INC. V
Diagnostic Cytopathology DOI 10.1002/dc
FINE-NEEDLE ASPIRATION CYTOLOGY OF METASTATIC PDS
Fig. 2. Papanicolaou-stained smear of FNA of lymph node metastasis exhibits markedly atypical and variably multinucleated polygonal and spindled cells (3400). [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]
abnormal mitoses. PDS/AFX is separated from UPS by the relatively rapid clinical presentation and the superficial, cutaneous origin of PDS/AFX in contrast to the deep-seated primary origin of UPS. Review of the literature reveals a few reports of AFXlike lesions that penetrate into the superficial subcutaneous component with frequent recurrences and widely disseminated metastases.6–12 It is these reports that prompted the development of the new clinicopathologic entity of PDS. In the FNA workup of a metastatic high-grade sarcoma, recognition of the above cytologic features and their association with the recently described PDS will prevent an unnecessary and potentially invasive search for more deeply seated sarcomas such as UPS, particularly in patients with a previous diagnosis of AFX. Allan R. Smith, M.D. Oleksandr Yergiyev, M.D. Jan F. Silverman, M.D. Department of Pathology and Laboratory Medicine Allegheny General Hospital Pittsburgh, Pennsylvania
References 1. McCalmont TH. AFX: What we now know. J Cutan Pathol 2011; 38:853–856. 2. McCalmont TH. Correction and clarification regarding AFX and pleomorphic dermal sarcoma. J Cutan Pathol 2012;39:8. 3. Miller K, Goodlad JR, Brenn T. Pleomorphic dermal sarcoma: Adverse histologic features predict aggressive behavior and allow distinction from atypical fibroxanthoma. Am J Surg Pathol 2012;36: 1317–1326. 4. Davidson JS, Demsey D. Atypical fibroxanthoma: Clinicopathologic determinants for recurrence and implications for surgical management. J Surg Oncol 2012;105:559–562. Fig. 3. High-power image of the excised ear lesion demonstrates numerous bizarre multinucleated malignant cells (Hematoxylin and Eosin, 3400). [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]
“benign” cutaneous histiocytoma (BCH).4,5 Immunohistochemical (IHC) studies for epithelial, stromal, and melanocytic lineages should readily identify the mesenchymal origin of PDS, BCH, and UPS and distinguish these from sarcomatoid carcinoma and melanoma.6,7 Unfortunately, there is no consistent IHC profile that is specific for PDS/ AFX, UPS, and BCH. Morphologically, BCH is composed of relatively uniform bland spindle cells with scattered aggregates of cells forming small fascicles, with rare to occasional mitotic figures. In contrast, the cells in PDS/AFX and UPS exhibit significant cytologic atypia with markedly pleomorphic cells containing profoundly atypical nuclei, prominent nucleoli, and numerous
5. Doyle LA, Fletcher CD. Metastasizing “benign” cutaneous fibrous histiocytoma: A clinicopathologic analysis of 16 cases. Am J Surg Pathol 2013;37:484–495. 6. Beer TW, Drury P, Heenan PJ. Atypical fibroxanthoma: A histological and immunohistochemical review of 171 cases. Am J Dermatopathol 2010;32:533–540. 7. Luzar B, Calonje E. Morphological and immunohistochemical characteristics of atypical fibroxanthoma with a special emphasis on potential diagnostic pitfalls: A review. J Cutan Pathol 2010;37:301– 309. 8. Fretzin DF, Helwig EB. Atypical fibroxanthoma of the skin. A clinicopathologic study of 140 cases. Cancer 1973;31:1541–1552. 9. Mirza B, Weedon D. Atypical fibroxanthoma: A clinicopathological study of 89 cases. Australas J Dermatol 2005;46:235–238. 10. Dahl I. Atypical fibroxanthoma of the skin. A clinicopathological study of 57 cases. Acta Pathol Microbiol Scand A 1976;84:183– 197. 11. Helwig EB, May D. Atypical fibroxanthoma of the skin with metastasis. Cancer 1986;57:368–376. 12. Jacobs DS, Edwards WD, Ye RC. Metastatic atypical fibroxanthoma of skin. Cancer 1975;35:457–463.
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