Pleomorphic Liposarcoma of the Scalp:

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1 198 Pleomorphic Liposarcoma of the Scalp: A Case Report and Literatures Review Sheng-Chi Chang, Hsin-Han Chen, Chao-I Wu, Erh-Kang Chou Meng-Shi Lin, Sophia Chia-Ning Chang Division of Plastic and Reconstructive Surgery, Department of Surgery, Medical University Hospital, Taichung, Taiwan Liposarcoma of the head and neck is quite rare. Only 17 cases of scalp liposarcoma have been reported before and none was ever reported in Taiwan. In this report, we present a case of pleomorphic liposarcoma of the scalp occurring in a 43-year-old woman. The case report highlights the high-grade histologic type of the scalp liposarcoma. The surgeons need more alert to lipoma-like tumors on the scalp and taking biopsy is always indicated. (J Plast Surg Asso R.O.C. 2008;17: ) Key words: liposarcoma, pleomorphic subtype, scalp Introduction Liposarcoma is the second most common soft tissue sarcoma. It often presents in patients of fifty and the common site including the thigh, buttock and retroperitoneum 1. However, liposarcoma of the head and neck is a rare entity that had only 200 reported cases since In these cases, there were only 17 cases occurring over the scalp 2. The other locations include oral cavity, larynx, orbital, neck and esophagus. Till now, there is no case reported of scalp liposarcoma in Taiwan. Therefore, we share our experience and the treatment result of a scalp liposarcoma in our association journal. Case Report A 43-year-old woman without systemic disease was admitted to our outpatient clinic for a 6 x 4 cm soft-tissue mass on the right parietal paramedical scalp that developed in recent 6months. At initial presentation, this mass was small, soft and painless. She ever consulted at local medical device where benign soft tissue mass was told. But the patient noted the tumor growing rapidly over the previous 6 months. So she visited to our outpatient clinic that physical examination revealed a well circumscribed, mobile, elastic and painless scalp mass. Her magnetic resonance image showed one 2.8 x 2.4 x 1.7 cm 3 heterogeneously hyperintensity nodule in the

2 Sheng-Chi Chang, Hsin-Han Chen, Chao-I Wu, Erh-Kang Chou, Meng-Shi Lin, Sophia Chia-Ning Chang 199 subcutaneous of the right parietal paramedical scalp. No significant osteolytic or osteosclerotic lesion is noted in the underlying calvarium (Fig 1). Systemic workup including chest X-ray and laboratory studies were all normal. The patient was scheduled for excision biopsy under general anesthesia. In the operating room, we found a well-defined heterogeneous firm mass about 4.5 x 3.5 x 1.5 cm 3 deep to galea layer of scalp after exploration. Histopathologic analysis reported pleomorphic liposarcoma of scalp with predominant rounded tumor cells and frequent abnormal mitosis (Fig 2). Wide excision with 5mm free margin was performed. The pericranium was also taken as a deep margin and had no tumor cells. The wound was primary closed with galea scoring (Fig 3). After operation, the patient was follow-up at out patient department. Fig. 1. A 28mm heterogenous tumor locates in the scalp of the right parietal paramedian; No significant osteolytic or osteosclerotic lesion is noted in the underlying calvarium. Fig. 2. A well-circumscribed tumor composed of round lipoblast-like cells with vacuolated cytoplasm and pleomorphic nuclei. Frequent abnormal mitosis is noted as well. The immunohistochemical study reveals positive for vimentin and S-100; negative for CK. Fig. 3. The wound was primary closed with galea scoring and the skin was closed with staplers. J.P.S.A.R.O.C Vol 17 No.2

3 200 Pleomorphic Liposarcoma of the Scalp Discussion The incident of liposarcoma is 9.8% to 16% of soft-tissue sarcoma that it s the second most common soft-tissue sarcoma next to malignant fibrous histocytoma 3. Although the definite etiology of liposarcoma is unknown, the relationship of trauma and subsequent hematoma proceeding tumor development has been reported. The period from the trauma to liposarcoma formation is often within one year 4. The survival from liposarcoma is much related to its histologic classification and location. The diagnosis of liposarcoma without histologic report does little in predicting the clinical behavior and prognosis of patients. For above reasons, the pathological classifications of liposarcoma have been ongoing change over the past years. Ewing was the first proposal in 1935 that he divided this tumor into two groups: myxoliposarcoma and granular cell liposarcoma 5. Stout divided it into three subtypes: well-differentiated myxoid, poordifferentiated myxoid and round cell 6. Enzinger and Winslow distinguished them into four types in 1962 that became the traditional World Health Organization classification, including welldifferentiated, myxoid, round cell and pleomorphic 7. The fifth variant, the dedifferentiated subtype, was also proposed latter and it is characterized by coexistence of well-differentiated liposarcoma with poorly differentiated, non-lipogenic tumor 2. Following the histological subtypes, the myxoid and the well-differentiated groups are considered low grade because they have a benign course with rare metastasis, indolent behavior and infrequent recurrence after complete resection. Some authors even question the metastatic ability of well-differentiated liposarcoma, leading the suggestion that they should be called atypical lipoma or atypical lipomatous tumor. However, the round cell and pleomorphic types are considered high grade that they have an increased local recurrence, metastatic and mortality rate. The prognosis for patients with pleomorphic or round-cell types is much less favorable than for patients with myxoid or well-differentiated liposarcoma: 5-year survival rate of 20% for the former compared with 80% for the latter 8. This can explain how important the definite histologic result from these patients. Myxoid liposarcoma is the most common subtype that presents 40 to 50 percent of all liposarcomas 3. It all occurred in adult, with peak incidence in the fifth decade of life. The low extremity, especially the thigh, is the most common site of myxoid tumor, followed by retroperitoneal origin. Cytogenetically, it is characterized by t(12;16)(q13;p11) abnormality. The round cell tumor also shares the same genetic mutation and it is considered a poorly differentiated type of myxoid tumor 9. The transition from myxoid to round cell tumor is associated with increasing in telomerase activity and c-myc protein expression. Thus, all myxoid tumor must be extensively sampled whether the local transformation to a round cell type, because this finding changes the clinical prognosis of this disease. Pleomorphic subtype represents 5% of liposarcomas and is a tumor of adulthood that occurs most commonly in the deep soft tissue of the extremities with the presentation of extensive necrosis. It behaves as a high-grade sarcoma that frequently metastasizes, most commonly to the lungs. Histologically, most pleomorphic liposarcomas are malignant fibrous histiocytoma [MFH]-like, differing by the presence of pleomorphic lipoblasts. According to the study of Katharine, that confirms the recurrence, metastasis and death due to pleomorphic liposarcoma occurred in 28%, 44% and 50% of 中華整形外科醫誌 : 民國 97 年 /17 卷 /2 期

4 Sheng-Chi Chang, Hsin-Han Chen, Chao-I Wu, Erh-Kang Chou, Meng-Shi Lin, Sophia Chia-Ning Chang 201 patients, respectively 10. Liposarcoma of the head and neck is rare. Niktaki and Nacimento ever reported eighty percent or more oral liposarcoma are welldifferentiated or myxoid type 11,12. Mandell at el also noted eighty-six percent of upper aerodigestive were well-differentiated or myxoid subtype 13. Despite these subtypes show a low metastatic rate, they do have a high local recurrent rate in head and neck. Proximity to vital structure and mistake in diagnosis of benign lipoma are the major reasons of high recurrence. Unlike other head and neck sites, the histological variety of scalp liposarcoma is quite different. We had reviewed previously reported scalp liposarcoma literatures and corrected 17 cases since 1943 (table 1). The WHO classification of reported cases of scalp liposarcoma include myxoid in 9 cases (53%), well-differentiated in 2 cases (11.7%), round cell in 2 cases (11.7%) and pleomorphic tumor in 4 cases (23.4%). The high grade histological subtypes occupy nearly half numbers and the die of liposarcoma occurs in 2 cases. This might be because they are arising in the skin, not in the deep soft tissue. It is accepted that liposarcoma exceedingly rare occurs in the subcutaneous soft tissue and the dermis. Dei et al ever report a series of subcutaneous liposarcoma and concluded they have an apparent tendency to show high-grade morphologic feature and have a predilection for the scalp 14. Besides, the delayed in the diagnosis of tumors in the hair-bearing scalp is a well-known problem, since they are hidden, painless and slowly growing. Therefore, the increased local recurrence of scalp liposarcoma could be expected. The diagnosis of liposarcoma is often difficulty by fine-needle aspiration or even open biopsy. Because the lipoblasts may be diffusely present in the tumor, a biopsy may not retrieve these cells. Often the diagnosis of liposarcoma is made after complete resection or on recurrence 15. Table 1 Reported Cases of Scalp Liposarcoma Author Year Age Sex Location Size WHO classification Therapy Disposition Stout et al M Temporal 8 cm Myxoid Excision NED 6.25 yrs Srivastava et al M Frontal? Myxoid?? Slullitel M Frontal? Round cell?? Enterline et al F Temperal 1 Well-differentiated Excision NED 6 yrs 46 M Occipital 15cm Myxoid Excision NED 12 yrs 61 F Temporal 9.5cm Well-differentiated Excision AWD 11 yrs Georgiades et al F Forehead 2cm Myxoid Excision NED 6 mo Hemmer et al F Occipitoparietal? Myxoid?? Sima et al F Temporal? Myxoid?? McCullough et al M Post-auricular 3 cm Myxoid Excision NED 2.5 yrs Herman et al M Parietal 12 cm Myxoid Excision Not report Kessler et al F Parietal 20 cm Pleomorphic Excision and XRT NED 2 yrs Dei Tos et al F? 1.5 cm Round cell Excision DOD 24mo 49 F? 3.5cm Pleomorphic Excision NED 7 mo 76 M? 19.5 cm Pleomorphic Excision Unknown 95 F? 12cm Pleomorphic None DUD (autopsy) Newlands et al F Parietal 7 cm Round cell Excision,Chemotherapy DOD 17 mo and XRT Present case F Parietal 5cm Pleomorphic Excision NED 4 mo NED, No evidence of disease; AWD, alive with disease; DUD, dead by unrelated disease; DOD, die of the disease; WHO, World Health Organization. J.P.S.A.R.O.C Vol 17 No.2

5 202 Pleomorphic Liposarcoma of the Scalp The radiographic appearance of liposarcoma varies from the histologic groups. The well-differentiated subtype typically has a homogenous appearance with sharp demarcation, similar to that of lipoma. The other histologic subtypes have a more heterogeneous appearance with soft tissue streaking and sometimes necrosis in the tumor. Many doctors publish magnetic resonance imaging because it can feature the lipomatous mass. Recent studies have shown the use of proton nuclear magnetic resonance spectroscopy may predict the histology of liposarcoma by the amount of triglyceride and phosphatidylcholine in the tumor. But it costs expensive 16. Nonsurgical treatment modalities are of limited use in liposarcoma. The standard treatment is wide excision. Frozen section should be done at the time of surgery to ensure the complete resection. Recurrence rate was from 17% to 80% if incomplete excision, as may occur when tumors are mistakenly believed to be benign lipomas 17. Lymph node dissection is not indicated unless there is concrete evidence of metastasis, which are very rare. Adjuvant radiotherapy is reserved for those cases in which the tumor could not be completely excised. But Pack and Pierson reported an increase in 5-year survival from 50% to 87% with combined surgery and radiation therapy compared to surgery alone 18. Chemotherapy may have efficacy in the treatment of unresectable disease or in an adjuvant setting for high-grade liposarcoma. Summary Liposarcoma of the scalp region is an extremely rare entity. It often presents a high-grade histologic type and has a risk factor to the patient because of delayed or misdiagnosis that results higher recurrence rate. The crucial treatment of this disease is complete excision. The surgeons need more alert to scalp lipoma-like tumors and take biopsy from all suction procedure. Reference 1. Herman O, Ariely D. Myxoid liposarcoma of the scalp: case report and literature review. Plast Reconstr Surg 1997;100: Shawn DN, Venu D. Mixed myxoid/round cell liposarcoma of the scalp. Am J Otolaryngol 2003; 24: Patel SR, Burgess MA, Plager C et al. Myxoid liposarcoma: Experience with chemotherapy. Cancer 1994;74: Enzinger FM, Winslow DP. Liposarcoma: A study of 103 cases. Virchows Arch 1962;335: McCulloch TM, Makielski KH, McNutt MA et al. Head and neck liposarcoma: A histopathologic reevaluation of reported cases. Arch Otolaryngol Head Neck Surg 1992;118: Chang HR, Hajdu SI, Collin C et al. The prognostic value of histologic subtypes in primary extremity liposarcoma. Cancer 1989;64: Alex k, Leonard B, Ephraim E et al. Liposarcoma of the scalp: A case report and review of the literature. Otol Head and Neck Surg 1997;117: Mro zek K, Szumigala J, Brooks JS et al. Round cell liposarcoma with the insertion (12;16) (q13;p11.2p13). Am J Clin Pathol 1997;108: Katharine AD, John RG, Elizabeth AM et al. Pleomorphic liposarcoma: A clinicopathologic analysis of 19 cases. Mod Pathol 2001;14: Nikitakis NG, Lopes MA, Pazoki AE et al. MDM2/CDK4/p53 oral liposarcoma: case report and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2001;92: Nascimento AF, McMenamin ME, Fletcher CD et al. Liposarcomas / atypical lipomatous tumors of the oral cavity: A clinicopathologic study of 23 中華整形外科醫誌 : 民國 97 年 /17 卷 /2 期

6 Sheng-Chi Chang, Hsin-Han Chen, Chao-I Wu, Erh-Kang Chou, Meng-Shi Lin, Sophia Chia-Ning Chang 203 cases. Ann Diagn Pathol 2002;6: Mandell DL, Brandwein MS, Woo P et al. Upper aerodigestive tract liposarcoma: Report on four cases and literature review. Laryngoscope 1999;109: Dei Tos AP, Mentzel T, Fletcher CD et al. Primary liposarcoma of the skin: a rare neoplasm with unusual high-grade features. Am J Dermatopathol 1998;20: Kilpatrick SE, Ward WG, Bos GD et al. The value of fine-needle aspiration biopsy in the differential diagnosis of adult myxoid sarcoma. Cancer 2000; 90: Millis K, Weybright P, Campbell N et al. Classification of human liposarcoma and lipoma using ex vivo proton NMR spectroscopy. Magn Reson Med 1999;41: Baldini EH, Goldberg J, Jenner C et al. Long-term outcomes after functional sparing surgery without radiation for soft tissue sarcoma. J Clin Oncol 1999;17: Stephen J, Mathes MD. Textbook of Plastic Surgery, second edition 2006;VII: Raaf JH, Ragsdale BD: Surgical management of liposarcoma. In: Bogumil GP, Fleegler EJ, eds. Tumors of the Hand and Upper Limb Marka RD, Edward WC. Liposarcoma of the tongue: case report and review of the literature. Head & Face Medicine 2006;2:21. Reprints request from: Sophia Chia-Ning Chang, MD, Ph.D. Department of Plastic Surgery, China Medical University Hospital School of Medicine, China Medical University Address: School of Medicine, China Medical University, Tel: Ext.1638 Fax: scnchang@pnc.com.tw J.P.S.A.R.O.C Vol 17 No.2

7 204 Pleomorphic Liposarcoma of the Scalp 頭皮惡性脂肪瘤 : 個案經驗分享及相關文獻整理 張伸吉林孟義陳信翰周爾康吳肇毅張家寧 頭頸部的惡性脂肪瘤非常少見, 尤其是頭皮原生的 根據歷年來的文獻統計只有 17 例, 在國內則從未被報導過 這個病例是一位 47 歲女性, 半年前發現一顆頭皮腫瘤, 但一直未被正確診斷, 直到來院經切片檢查後才發現是惡性脂肪瘤 經開刀切除後, 病患順利出院並持續門診追蹤 這篇文章主要是探討頭皮惡性脂肪瘤的特異以及分類 治療和預後 中華整形外科醫誌 : 民國 97 年 /17 卷 /2 期