AJCP / Case Report
Composite Hemangioendothelioma An Unusual Presentation of a Rare Vascular Tumor Rod Mahmoudizad, MD,1,2 Aman Samrao, MD,1 Jason J. Bentow, MD,1 Shi-Kaung Peng, MD, PhD,3 and Neil Bhatia, MD, FAAD1
CME/SAM
From the 1Division of Dermatology and 3Department of Pathology, Harbor-UCLA Medical Center, Torrance, CA; and 2Department of Dermatology, Texas A&M HSC COM-Scott and White, Temple, TX Key Words: Composite hemangioendothelioma; Vascular; Metastasis Am J Clin Pathol May 2014;141:732-736 DOI: 10.1309/AJCPXEK50YPRNDHX
ABSTRACT Objectives: Composite hemangioendothelioma (CHE) has been recently recognized as a low- to intermediate-grade vascular tumor. CHEs are rare vascular tumors that are clinically similar to more common vascular tumors but histologically exhibit a composite of hemangioendothelioma variants. We report the first case of a CHE on the scalp and the fifth case to show findings supportive of regional metastasis. Methods: Our patient had a multilobulated, violaceous scalp nodule, which on histologic examination revealed epithelioid, retiform, and spindle-cell components and rare foci of intermediate-grade mitotic activity consistent with CHE. Results: Imaging studies were performed and revealed an abnormal uptake of contrast media in a posterior neck nodule that, when examined via fine-needle aspiration, revealed clumps of atypical cells. Conclusions: This unique case presentation is representative of the variability seen in the presentation of CHE and highlights the importance of considering CHE on the clinical and histologic differential of vascular tumors.
732 732
Am J Clin Pathol 2014;141:732-736 DOI: 10.1309/AJCPXEK50YPRNDHX
Upon completion of this activity you will be able to: • describe the components that make up a composite hemangioendothelioma. • describe the clinical features associated with this rarely reported vascular neoplasm. • identify the appropriate histochemical markers associated with hemangioendotheliomas. The ASCP is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The ASCP designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 Credit ™ per article. Physicians should claim only the credit commensurate with the extent of their participation in the activity. This activity qualifies as an American Board of Pathology Maintenance of Certification Part II Self-Assessment Module. The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose. Questions appear on p 756. Exam is located at www.ascp.org/ajcpcme.
The entity of composite hemangioendothelioma (CHE) has been recognized as a low- to intermediate-grade malignant vascular tumor displaying varying combinations of benign and malignant vascular components.1 Histopathologic diagnosis requires the presence of at least two hemangioendothelioma variants. Epithelioid and retiform variants are most commonly observed, but foci of spindle-cell hemangioma may also be present. Angiosarcoma-like foci are commonly identified within the tumor. To our knowledge, 28 cases of CHE with all three of these hemangioendothelioma variants have been reported in the worldwide English literature.1-18 Herein, we report the second case of a CHE with epithelioid, retiform, and spindle-cell hemangioma components on the scalp and the fifth case of CHE overall to show findings supportive of regional metastasis. © American Society for Clinical Pathology
AJCP / Case Report
Case Report A 68-year-old man presented with a posttraumatic 10-month history of a growing nodule on the vertex of his scalp. The nodule became painful and pruritic and started to enlarge. It was diagnosed as a bacterial infection and treated with two courses of amoxicillin-clavulanate. Six months later, he presented to our clinic with continued growth of the scalp lesion and persistent pain and pruritus. On examination, a large, multilobulated, violaceous nodule involving the vertex of the scalp measured 6.3 × 5.3 cm ❚Image 1A❚ with four adjacent surrounding skin-colored papules each measuring 1.3 × 1 cm, 1 × 1 cm, 0.8 × 0.8 cm, and 0.5 × 0.5 cm. On further examination, the patient was also found to have a small subcutaneous nodule in the right posterior neck. Histologic examination revealed a vascular tumor with occasional blister cells and a complex combination of histologic patterns consisting of spindle-cell hemangioma, retiform hemangioendothelioma, and epithelioid hemangioendothelioma components, suggestive of CHE ❚Image 1B❚, ❚Image 2A❚, ❚Image 2B❚, and ❚Image 2C❚. The spindle-cell hemangioma component is identified by the presence of thin-walled cavernous channels that may contain thrombi or phleboliths, solid areas of spindle cells with slit-like vascular spaces, and plump endothelial cells, either in groups or lining vascular channels, that may have intracytoplasmic vacuoles (Image 2C).19 The retiform hemangioendothelioma component is identified by the pattern of arborizing vessels lined by “hobnail,” monomorphous endothelial cells with scant cytoplasm and minimal or no atypia, along with prominent lymphocytic infiltrate, and
A
lymphocytes within the lumina of vessels, closely apposed to endothelial cells (Image 2B).20 The epithelioid hemangioendothelioma component is identified by the presence of a proliferation of nests and cords of plump, epithelioid to spindle-shaped endothelial cells in a fibromyxoid stroma that may contain cytoplasmic vacuoles resembling a signet ring (Image 2A).21 Immunohistochemistry (IHC) stains of tumor cells were positive for CD31 ❚Image 2D❚, factor VIII, and vimentin with rare D2-40–positive foci, supporting a neoplasm of vascular origin. The tumor cells were negative for a-actin, desmin, KP1, AE1/3, S100, CD34, and factor XIIIa, as well as human herpesvirus 8. There were focal areas of atypical and mitotically active cells ❚Image 3A❚. Ki-67 immunostain revealed a 20% proliferative rate consistent with a tumor of intermediate malignant potential ❚Image 3B❚. A computed tomography (CT) scan of the head and neck showed a multilobulated vertex scalp mass that did not extend intracranially. A fluorodeoxyglucose (FDG) positron emission tomography (PET) scan showed abnormal uptake of FDG in the superior scalp, corresponding to the patient’s large scalp lesion, as well as abnormal asymmetric areas of uptake in the posterior soft tissues of the neck and in the region of the left deep parotid gland, consistent with regional adenopathy. Fine-needle aspiration of the right posterior neck nodule showed a few clusters of atypical cells in a background of abundant blood ❚Image 4❚. A CD31 stain performed on alcohol-fixed tissue was negative. However, due to the inherent false-negative rate of performing IHC for formalin-fixed tissue on alcohol-fixed tissues, these
B
❚Image 1❚ A, Multilobulated violaceous plaque on scalp. B, Low-power histologic examination revealed a neoplastic process with a nodular growth pattern in the dermis (H&E, ×40). The asterisk indicates the biopsy site. © American Society for Clinical Pathology
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results were determined to be noncontributory. Based on the cytologic appearance, correlated with increased FDG uptake on the PET scan, a diagnosis of regional metastasis was made. The patient was referred to head and neck surgery for wide local excision of the scalp lesion. However, due to cardiovascular disease, the patient was a poor surgical candidate. The tumor was treated with radiation, which resulted in tumor shrinkage, and the patient was then lost to follow-up.
Discussion CHE has been described as a neoplasm with wide variability and can mimic other vascular tumors both histopathologically and clinically. There is a wide variability in the age of the patients diagnosed with CHE, from newborns to the elderly. Most of these tumors have been found on the distal extremities. Exceptions include the tongue,1 mandibular vestibule,6 Achilles tendon,4 axilla,5 thigh,6,12 upper arm,6 cheek mucosa,8 upper back,10 mediastinum,11 hypopharynx,14 scalp,16 nose,17 and kidney.18 Local recurrence of these
A
B
C
D
❚Image 2❚ Higher power revealed a vascular tumor with a heterogeneous pattern containing three distinct components. A, The epithelioid component composed of epithelioid endothelial cells and the diagnostic “blister cells”: endothelial cells with intracytoplasmic vacuoles (arrow; H&E, ×200) . B, The retiform component composed of arborizing vessels lined by “hobnail” endothelial cells (arrow), interstitial lymphocytes, and an abundance of RBCs (H&E, ×200). C, The spindle cell component composed of solid areas of spindle cells (arrow) with slit-like vascular spaces (H&E, ×200). D, A positive immunoperoxidase stain for CD31 in tumor cells (×100) supports an endothelial origin. 734 734
Am J Clin Pathol 2014;141:732-736 DOI: 10.1309/AJCPXEK50YPRNDHX
© American Society for Clinical Pathology
AJCP / Case Report
tumors after initial excision has occurred in eight of the previously reported 28 cases, while metastasis is even rarer, occurring in four cases reported thus far. Previously described CHEs have been observed anywhere from a few months to several years prior to presentation. Our patient observed the neoplasm for a period of less than a year following a trauma to the scalp, again signaling the possibility of a potentially aggressive tumor. Whether or not the trauma was a causative factor for the neoplasm or simply a means to first observe it is unclear, however, as trauma prior to the appearance of CHE has not been published previously. This unique presentation is another example of the variability seen in the presentation of CHE and another reason to consider CHE on the clinical and histopathologic differential for vascular neoplasms. Based on the clinical findings and history, the neoplasm observed in our case was initially concerning for angiosarcoma; however, the predominant components of spindle-cell hemangioma and epithelioid and retiform hemangioendothelioma on histopathology confirmed the diagnosis of CHE. Although the proliferative index was 20% based on staining with Ki-67, the diagnosis of angiosarcoma was excluded due to the rare foci of angiosarcomalike components in the tumor. Ki-67 has been found to be positive in hemangioendotheliomas. In a study performed by Hisaoka et al,22 12 cases of spindle-cell hemangioma were stained for Ki-67 and had a range of expression from 0.1% to 14.9%. However, to our knowledge, no case series, reviews, or case reports are available analyzing the utility of Ki-67 staining in differentiating angiosarcoma from CHEs. Whether the tumor represents an unknown variant of angiosarcoma remains to be determined.
A
Given a history of potential metastasis related to this relatively newly described tumor, further investigation has been warranted and confirmed regional adenopathy and favored metastasis when correlated with imaging, although cytology was noncontributory.7 Although metastasis is rare, this case suggests that evaluation of the regional lymph nodes using ultrasound, CT, or magnetic resonance imaging preoperatively and at periodic intervals may be useful. The satellite
❚Image 4❚ Fine-needle aspiration of the right neck mass visualized on computed tomography showed clumps of atypical endothelial cells in a background of RBCs, supporting the malignant nature of this tumor (Papanicolaou, ×400). CD31 was noncontributory.
B
❚Image 3❚ A, Biopsy of the primary lesion showed multiple mitotic figures and atypical nuclei in select focal areas (H&E, ×400). B, A Ki-67 stain of the primary lesion showed an intermediate proliferative rate (20%) (×100). © American Society for Clinical Pathology
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nodules in the vicinity of the primary hemangioendothelioma may represent cutaneous metastases as described by Requena et al,7 indicating a more aggressive tumor. Although the best therapeutic approach and prognosis remain unclear, due to the paucity of cases in the literature, the reported high rate of local recurrence suggests excision should extend beyond clinical margins. Adjuvant radiation therapy and chemotherapy may also be considered, although more data are needed to obtain a better understanding of prognosis and malignant potential of this rare vascular tumor.13 Address reprint requests to Dr Mahmoudizad: Dept of Dermatology, Texas A&M HSC COM-Scott and White, 409 W Adams, Temple, TX 76501; [email protected].
References 1. Nayler SJ, Rubin BP, Calonje E, et al. Composite haemangioendothelioma: a complex, low-grade vascular lesion mimicking angiosarcoma. Am J Surg Pathol. 2000;24:352-361. 2. Reis-Filho JS, Paiva ME, Lopes JM. Congenital composite hemangioendothelioma: case report and reappraisal of the hemangioendothelioma spectrum. J Cutan Pathol. 2002;29:2226-2231. 3. Biagioli M, Sbano P, Miracco C, et al. Composite cutaneous haemangioendothelioma: case report and review of the literature. Clin Exp Dermatol. 2005;30:385-387. 4. Tronnier M, Vogelbruch M, Kutzner H. Spindle cell hemangioma and epithelioid hemangioendothelioma arising in an area of lymphedema. Am J Dermatopathol. 2006;28:223227. 5. Chu YC, Choi SJ, Park IS, et al. Composite hemangioendothelioma: a case report. Korean J Pathol. 2006;40:142-147. 6. Fukunaga M, Suzuki K, Saegusa N, et al. Composite hemangioendothelioma: report of 5 cases including one with associated Maffucci syndrome. Am J Surg Pathol. 2007;31:1567-1572. 7. Requena L, Luis Diaz J, Manzarbeitia F, et al. Cutaneous composite hemangioendothelioma with satellitosis and lymph node metastases. J Cutan Pathol. 2008;35:225-230. 8. Fasolis M, Iaquinta C, Montesco MC, et al. Composite hemangioendothelioma of the oral cavity: case report and review of the literature. Head Neck. 2008;30:974-979.
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9. Utas S, Canöz O, Ferahbas A, et al. Composite cutaneous hemangioendothelioma treated with interferon. J Eur Acad Dermatol Venereol. 2008;22:503-505. 10. Tejera-Vaquerizo A, Herrera-Ceballos E, Bosch-García R, et al. Composite cutaneous hemangioendothelioma on the back. Am J Dermatopathol. 2008;30:262-264. 11. Cakir E, Demirag F, Gulhan E, et al. Mediastinal composite hemangioendothelioma: a rare tumor at an unusual location. Tumori. 2009;95:98-100. 12. Aydingöz IE, Demirkesen C, Serdar ZA, et al. Composite haemangioendothelioma with lymph-node metastasis: an unusual presentation at an uncommon site. Clin Exp Dermatol. 2009;34:e802-e806. 13. Stratton JS, Billings SD. Vascular tumors of intermediate malignancy: a review and update. Dermatol Sinica. 2009;27:140-153. 14. Tsai JW, Huang HY, Lee JC, et al. Composite haemangioendothelioma: report of four cases with emphasis on atypical clinical presentation. Pathology. 2011;43:176-180. 15. McNab PM, Quigley BC, Glass LF, et al. Composite hemangioendothelioma and its classification as a low-grade malignancy. Am J Dermatopathol. 2013;35:517-522. 16. Liau JY, Lee FY, Chiu CS, et al. Composite hemangioendothelioma presenting as a scalp nodule with alopecia. J Am Acad Dermatol. 2013;69:e98-e99. 17. Tateishi J, Saeki H, Ito K, et al. Cutaneous composite hemangioendothelioma on the nose treated with electron beam. Int J Dermatol. 2013;52:1618-1619. 18. Zhang J, Wu B, Zhou GQ, et al. Composite hemangioendothelioma arising from the kidney: case report with review of the literature. Int J Clin Exp Pathol. 2013;6:1935-1941. 19. Weiss SW, Enzinger FM. Spindle cell hemangioendothelioma: a low-grade angiosarcoma resembling a cavernous hemangioma and Kaposi’s sarcoma. Am J Surg Pathol. 1986;10:521-530. 20. Calonje E, Fletcher CDM, Wilson-Jones E, et al. Retiform hemangioendothelioma: a distinctive form of low-grade angiosarcoma delineated in a series of 15 cases. Am J Surg Pathol. 1994;18:115-125. 21. Weiss SW, Enzinger FM. Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma. Cancer. 1982;50:970-981. 22. Hisaoka M, Kouho H, Aoki T, et al. DNA flow cytometric and immunohistochemical analysis of proliferative activity in spindle cell haemangioendothelioma. Histopathology. 1995:27:451-456.
© American Society for Clinical Pathology
Composite Hemangioendothelioma An Unusual Presentation of a Rare Vascular Tumor Rod Mahmoudizad, MD,1,2 Aman Samrao, MD,1 Jason J. Bentow, MD,1 Shi-Kaung Peng, MD, PhD,3 and Neil Bhatia, MD, FAAD1
CME/SAM
From the 1Division of Dermatology and 3Department of Pathology, Harbor-UCLA Medical Center, Torrance, CA; and 2Department of Dermatology, Texas A&M HSC COM-Scott and White, Temple, TX Key Words: Composite hemangioendothelioma; Vascular; Metastasis Am J Clin Pathol May 2014;141:732-736 DOI: 10.1309/AJCPXEK50YPRNDHX
ABSTRACT Objectives: Composite hemangioendothelioma (CHE) has been recently recognized as a low- to intermediate-grade vascular tumor. CHEs are rare vascular tumors that are clinically similar to more common vascular tumors but histologically exhibit a composite of hemangioendothelioma variants. We report the first case of a CHE on the scalp and the fifth case to show findings supportive of regional metastasis. Methods: Our patient had a multilobulated, violaceous scalp nodule, which on histologic examination revealed epithelioid, retiform, and spindle-cell components and rare foci of intermediate-grade mitotic activity consistent with CHE. Results: Imaging studies were performed and revealed an abnormal uptake of contrast media in a posterior neck nodule that, when examined via fine-needle aspiration, revealed clumps of atypical cells. Conclusions: This unique case presentation is representative of the variability seen in the presentation of CHE and highlights the importance of considering CHE on the clinical and histologic differential of vascular tumors.
732 732
Am J Clin Pathol 2014;141:732-736 DOI: 10.1309/AJCPXEK50YPRNDHX
Upon completion of this activity you will be able to: • describe the components that make up a composite hemangioendothelioma. • describe the clinical features associated with this rarely reported vascular neoplasm. • identify the appropriate histochemical markers associated with hemangioendotheliomas. The ASCP is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The ASCP designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 Credit ™ per article. Physicians should claim only the credit commensurate with the extent of their participation in the activity. This activity qualifies as an American Board of Pathology Maintenance of Certification Part II Self-Assessment Module. The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose. Questions appear on p 756. Exam is located at www.ascp.org/ajcpcme.
The entity of composite hemangioendothelioma (CHE) has been recognized as a low- to intermediate-grade malignant vascular tumor displaying varying combinations of benign and malignant vascular components.1 Histopathologic diagnosis requires the presence of at least two hemangioendothelioma variants. Epithelioid and retiform variants are most commonly observed, but foci of spindle-cell hemangioma may also be present. Angiosarcoma-like foci are commonly identified within the tumor. To our knowledge, 28 cases of CHE with all three of these hemangioendothelioma variants have been reported in the worldwide English literature.1-18 Herein, we report the second case of a CHE with epithelioid, retiform, and spindle-cell hemangioma components on the scalp and the fifth case of CHE overall to show findings supportive of regional metastasis. © American Society for Clinical Pathology
AJCP / Case Report
Case Report A 68-year-old man presented with a posttraumatic 10-month history of a growing nodule on the vertex of his scalp. The nodule became painful and pruritic and started to enlarge. It was diagnosed as a bacterial infection and treated with two courses of amoxicillin-clavulanate. Six months later, he presented to our clinic with continued growth of the scalp lesion and persistent pain and pruritus. On examination, a large, multilobulated, violaceous nodule involving the vertex of the scalp measured 6.3 × 5.3 cm ❚Image 1A❚ with four adjacent surrounding skin-colored papules each measuring 1.3 × 1 cm, 1 × 1 cm, 0.8 × 0.8 cm, and 0.5 × 0.5 cm. On further examination, the patient was also found to have a small subcutaneous nodule in the right posterior neck. Histologic examination revealed a vascular tumor with occasional blister cells and a complex combination of histologic patterns consisting of spindle-cell hemangioma, retiform hemangioendothelioma, and epithelioid hemangioendothelioma components, suggestive of CHE ❚Image 1B❚, ❚Image 2A❚, ❚Image 2B❚, and ❚Image 2C❚. The spindle-cell hemangioma component is identified by the presence of thin-walled cavernous channels that may contain thrombi or phleboliths, solid areas of spindle cells with slit-like vascular spaces, and plump endothelial cells, either in groups or lining vascular channels, that may have intracytoplasmic vacuoles (Image 2C).19 The retiform hemangioendothelioma component is identified by the pattern of arborizing vessels lined by “hobnail,” monomorphous endothelial cells with scant cytoplasm and minimal or no atypia, along with prominent lymphocytic infiltrate, and
A
lymphocytes within the lumina of vessels, closely apposed to endothelial cells (Image 2B).20 The epithelioid hemangioendothelioma component is identified by the presence of a proliferation of nests and cords of plump, epithelioid to spindle-shaped endothelial cells in a fibromyxoid stroma that may contain cytoplasmic vacuoles resembling a signet ring (Image 2A).21 Immunohistochemistry (IHC) stains of tumor cells were positive for CD31 ❚Image 2D❚, factor VIII, and vimentin with rare D2-40–positive foci, supporting a neoplasm of vascular origin. The tumor cells were negative for a-actin, desmin, KP1, AE1/3, S100, CD34, and factor XIIIa, as well as human herpesvirus 8. There were focal areas of atypical and mitotically active cells ❚Image 3A❚. Ki-67 immunostain revealed a 20% proliferative rate consistent with a tumor of intermediate malignant potential ❚Image 3B❚. A computed tomography (CT) scan of the head and neck showed a multilobulated vertex scalp mass that did not extend intracranially. A fluorodeoxyglucose (FDG) positron emission tomography (PET) scan showed abnormal uptake of FDG in the superior scalp, corresponding to the patient’s large scalp lesion, as well as abnormal asymmetric areas of uptake in the posterior soft tissues of the neck and in the region of the left deep parotid gland, consistent with regional adenopathy. Fine-needle aspiration of the right posterior neck nodule showed a few clusters of atypical cells in a background of abundant blood ❚Image 4❚. A CD31 stain performed on alcohol-fixed tissue was negative. However, due to the inherent false-negative rate of performing IHC for formalin-fixed tissue on alcohol-fixed tissues, these
B
❚Image 1❚ A, Multilobulated violaceous plaque on scalp. B, Low-power histologic examination revealed a neoplastic process with a nodular growth pattern in the dermis (H&E, ×40). The asterisk indicates the biopsy site. © American Society for Clinical Pathology
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results were determined to be noncontributory. Based on the cytologic appearance, correlated with increased FDG uptake on the PET scan, a diagnosis of regional metastasis was made. The patient was referred to head and neck surgery for wide local excision of the scalp lesion. However, due to cardiovascular disease, the patient was a poor surgical candidate. The tumor was treated with radiation, which resulted in tumor shrinkage, and the patient was then lost to follow-up.
Discussion CHE has been described as a neoplasm with wide variability and can mimic other vascular tumors both histopathologically and clinically. There is a wide variability in the age of the patients diagnosed with CHE, from newborns to the elderly. Most of these tumors have been found on the distal extremities. Exceptions include the tongue,1 mandibular vestibule,6 Achilles tendon,4 axilla,5 thigh,6,12 upper arm,6 cheek mucosa,8 upper back,10 mediastinum,11 hypopharynx,14 scalp,16 nose,17 and kidney.18 Local recurrence of these
A
B
C
D
❚Image 2❚ Higher power revealed a vascular tumor with a heterogeneous pattern containing three distinct components. A, The epithelioid component composed of epithelioid endothelial cells and the diagnostic “blister cells”: endothelial cells with intracytoplasmic vacuoles (arrow; H&E, ×200) . B, The retiform component composed of arborizing vessels lined by “hobnail” endothelial cells (arrow), interstitial lymphocytes, and an abundance of RBCs (H&E, ×200). C, The spindle cell component composed of solid areas of spindle cells (arrow) with slit-like vascular spaces (H&E, ×200). D, A positive immunoperoxidase stain for CD31 in tumor cells (×100) supports an endothelial origin. 734 734
Am J Clin Pathol 2014;141:732-736 DOI: 10.1309/AJCPXEK50YPRNDHX
© American Society for Clinical Pathology
AJCP / Case Report
tumors after initial excision has occurred in eight of the previously reported 28 cases, while metastasis is even rarer, occurring in four cases reported thus far. Previously described CHEs have been observed anywhere from a few months to several years prior to presentation. Our patient observed the neoplasm for a period of less than a year following a trauma to the scalp, again signaling the possibility of a potentially aggressive tumor. Whether or not the trauma was a causative factor for the neoplasm or simply a means to first observe it is unclear, however, as trauma prior to the appearance of CHE has not been published previously. This unique presentation is another example of the variability seen in the presentation of CHE and another reason to consider CHE on the clinical and histopathologic differential for vascular neoplasms. Based on the clinical findings and history, the neoplasm observed in our case was initially concerning for angiosarcoma; however, the predominant components of spindle-cell hemangioma and epithelioid and retiform hemangioendothelioma on histopathology confirmed the diagnosis of CHE. Although the proliferative index was 20% based on staining with Ki-67, the diagnosis of angiosarcoma was excluded due to the rare foci of angiosarcomalike components in the tumor. Ki-67 has been found to be positive in hemangioendotheliomas. In a study performed by Hisaoka et al,22 12 cases of spindle-cell hemangioma were stained for Ki-67 and had a range of expression from 0.1% to 14.9%. However, to our knowledge, no case series, reviews, or case reports are available analyzing the utility of Ki-67 staining in differentiating angiosarcoma from CHEs. Whether the tumor represents an unknown variant of angiosarcoma remains to be determined.
A
Given a history of potential metastasis related to this relatively newly described tumor, further investigation has been warranted and confirmed regional adenopathy and favored metastasis when correlated with imaging, although cytology was noncontributory.7 Although metastasis is rare, this case suggests that evaluation of the regional lymph nodes using ultrasound, CT, or magnetic resonance imaging preoperatively and at periodic intervals may be useful. The satellite
❚Image 4❚ Fine-needle aspiration of the right neck mass visualized on computed tomography showed clumps of atypical endothelial cells in a background of RBCs, supporting the malignant nature of this tumor (Papanicolaou, ×400). CD31 was noncontributory.
B
❚Image 3❚ A, Biopsy of the primary lesion showed multiple mitotic figures and atypical nuclei in select focal areas (H&E, ×400). B, A Ki-67 stain of the primary lesion showed an intermediate proliferative rate (20%) (×100). © American Society for Clinical Pathology
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Mahmoudizad et al / Composite Hemangioendothelioma
nodules in the vicinity of the primary hemangioendothelioma may represent cutaneous metastases as described by Requena et al,7 indicating a more aggressive tumor. Although the best therapeutic approach and prognosis remain unclear, due to the paucity of cases in the literature, the reported high rate of local recurrence suggests excision should extend beyond clinical margins. Adjuvant radiation therapy and chemotherapy may also be considered, although more data are needed to obtain a better understanding of prognosis and malignant potential of this rare vascular tumor.13 Address reprint requests to Dr Mahmoudizad: Dept of Dermatology, Texas A&M HSC COM-Scott and White, 409 W Adams, Temple, TX 76501; [email protected].
References 1. Nayler SJ, Rubin BP, Calonje E, et al. Composite haemangioendothelioma: a complex, low-grade vascular lesion mimicking angiosarcoma. Am J Surg Pathol. 2000;24:352-361. 2. Reis-Filho JS, Paiva ME, Lopes JM. Congenital composite hemangioendothelioma: case report and reappraisal of the hemangioendothelioma spectrum. J Cutan Pathol. 2002;29:2226-2231. 3. Biagioli M, Sbano P, Miracco C, et al. Composite cutaneous haemangioendothelioma: case report and review of the literature. Clin Exp Dermatol. 2005;30:385-387. 4. Tronnier M, Vogelbruch M, Kutzner H. Spindle cell hemangioma and epithelioid hemangioendothelioma arising in an area of lymphedema. Am J Dermatopathol. 2006;28:223227. 5. Chu YC, Choi SJ, Park IS, et al. Composite hemangioendothelioma: a case report. Korean J Pathol. 2006;40:142-147. 6. Fukunaga M, Suzuki K, Saegusa N, et al. Composite hemangioendothelioma: report of 5 cases including one with associated Maffucci syndrome. Am J Surg Pathol. 2007;31:1567-1572. 7. Requena L, Luis Diaz J, Manzarbeitia F, et al. Cutaneous composite hemangioendothelioma with satellitosis and lymph node metastases. J Cutan Pathol. 2008;35:225-230. 8. Fasolis M, Iaquinta C, Montesco MC, et al. Composite hemangioendothelioma of the oral cavity: case report and review of the literature. Head Neck. 2008;30:974-979.
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