脂肪瘤样多形性腺瘤- 罕见病例

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Dr.小院养兰上传了一例非常罕见的“梭形细胞脂肪瘤+腺体的肿瘤”。Dr. Fangg 诊断为脂肪瘤样多形性腺瘤。此病例非常好！请大家注意。因为主题被锁定，只好发此件，抱歉！

现将该病例移至《疑难罕见病例专栏》！请各位老师继续讨论！可以拖动本楼内网页右侧的拖条上下浏览该病例！

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脂肪瘤样多形性腺瘤

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1 楼发表于2010-04-17 18:00:00举报|引用
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以下是引用xljin8在2010-4-17 17:59:00的发言：

非常好的病例！同意Dr.Fangg的诊断：脂肪瘤样多形性腺瘤。自1999年 Seifert首例报道至今，文献报道病例不到10例，国内尚未见报道。

1. Kondo T. A case of lipomatous pleomorphic adenoma in the parotid gland: a case report. Diagn Pathol. 2009;4:16.

Division of Pathology, Department of Pathology, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-cho, Chuo-ku, Kobe 650-0017, Japan.

INTRODUCTION: Pleomorphic adenoma is the most common benign neoplasm of the salivary glands. Extensive lipomatous involvement of the tumor is, however, a very rare finding. CASE REPORT: Herein, a rare case of lipomatous pleomorphic adenoma arising in the parotid gland of a 14-year-old Japanese woman is presented. CONCLUSION: This is the sixth case of lipomatous pleomorphic adenoma in the English literature. Recognition of this rare subtype of pleomorphic adenoma is important for clinical diagnosis and management. On CT scan, it may not be detected possibly due to the extensive fatty component.

2. Leroy X, Wacrenier A, Augusto D, Leteurtre E, Desaulty A, Gosselin B. [Lipomatous pleomorphic adenoma of the parotid] Ann Pathol. 2002 Jun;22(3):219-21.

Service d'Anatomie et de Cytologie Pathologiques, pôle Huriez, CHRU, 59045 Lille,France. x-leroy@chru-lille.fr

Lipomatous tumors rarely occur in the salivary glands. We report an unusual case of lipomatous pleomorphic adenoma in a 47-year-old man. The patient had no significant medical history and presented with a well circumscribed nodule measuring 3 cm in the right parotid. Histologically, the tumour was predominantly composed of sheets of mature fat cells. Rare myoepithelial cells and exceptional tubules were intermingled with the mature adipose tissue. One year after surgery

the patient was alive without recurrence. In conclusion, it is a rare neoplasm who needs to be recognized and discussed with true fatty tumors and lipomatosis.

3. Korkmaz H, Adsay NV, Jacobs JR, Lucas DR. Lipomatous pleomorphic adenoma of parotid gland. Histopathology. 2002;40:487-8.

4. Skálová A, Stárek I, Simpson RH, Kucerová V, Dvorácková J, Curík R, Dusková M. Spindle cell myoepithelial tumours of the parotid gland with extensive lipomatous metaplasia. A report of four cases with immunohistochemical and ultrastructural findings. Virchows Arch. 2001;439:762-7.

We report four cases of parotid gland tumours composed predominantly of spindle-shaped myoepithelial cells and mature adipocytes. The central portion of one tumour showed extensive adipose differentiation, whereas in the peripheral parts there were small foci of ductal epithelium arranged in cords and tubules within an abundant myxoid stroma. The other cases were adipose spindle cell myoepitheliomas without an obvious glandular component. Under high-power examination, a transition between modified spindle-shaped myoepithelial cells and adipocytes was observed, and this was confirmed with immunohistochemistry. Ultrastructurally, the modified myoepithelial cells showed intracytoplasmic tonofilaments, bundles of actin microfilaments and lipid droplets. A possible pathogenesis is proposed of true metaplastic transformation of myoepithelial cells to adipocytes. This lesion is important to identify correctly, as inadequate surgery can lead to recurrence.

5.Seifert G, Donath K, Schäfer R. Lipomatous pleomorphic adenoma of the parotid gland. Classification of lipomatous tissue in salivary glands.

Pathol Res Pract. 1999;195:247-52.

Institute of Pathology, University of Hamburg, Germany.

Lipomatous pleomorphic adenoma is an unusual subtype with a lipomatous stromal component of more than 90% of the tumour tissue. This special type of pleomorphic adenoma must be distinguished from other types of lipomatous tumours or non-tumourous lipomatosis of the salivary glands. Until now only two cases of lipomatous pleomorphic adenoma have been reported in the literature. We report of a 36-year old woman who developed a well circumscribed nodule measuring 3.5 x 2.5 x 2 cm in the right parotid gland. The cut surface was grey-yellowish. Histologically, more than 90% of the tumour tissue was fatty tissue with univacuolar adipocytes. The pleomorphic epithelial elements were duct-like cells forming small lumina and spindle-shaped myoepithelial cell with surrounding mucoid stroma. Components of pleomorphic adenoma were intermingled with mature adipose tissue which was more concentrated in the central portion of the adenoma.Some compressed epithelial cords in the adipose tissue formed a septa-like pattern. The differential diagnosis to other lipomatous tumours (lipoadenoma,lipoma) and to non-tumourous interstitial lipomatosis as well as the possible pathogenesis as metaplastic change or epithelial-mesenchymal transdifferentiation are discussed.