LeBoit P E, Barr R J, Burall S, Metcalf J S, Yen T S, Wick M R
Department of Pathology, University of California, San Francisco 94143-0506.
Am J Surg Pathol. 1991 Jan;15(1):48-58. doi: 10.1097/00000478-199101000-00006.
Most cutaneous and noncutaneous granular-cell tumors are currently thought to be of Schwann-cell derivation. We present seven unusual cutaneous granular-cell lesions in which Schwann-cell origin can be excluded or is inapparent. Four of these lesions are of a previously undescribed type, and, unlike conventional granular-cell tumors of the skin, show a polypoid configuration, numerous mitoses, cytologic atypia, and a primitive immunophenotype. We propose the term "primitive polypoid granular-cell tumor" for these lesions. One occurred in a child, and three in adults. There have been no metastases to date, with follow-up periods of 2, 4, 4, and 16 years, respectively, although one tumor recurred locally. Additional cases and longer follow-up may be required to rule out the possibility that primitive polypoid granular-cell tumor is a low-grade malignancy. Two other granular-cell lesions represent variants of leiomyosarcoma, one of which widely metastasized. The last case is a granular-cell form of nodular basal-cell carcinoma. Cutaneous granular-cell neoplasms can show varying differentiation and behavior. Pathologists should not equate the occurrence of cytoplasmic granularity in a cutaneous neoplasm with the diagnosis of granular-cell schwannoma.
目前认为，大多数皮肤和非皮肤颗粒细胞瘤起源于施万细胞。我们报告了7例不寻常的皮肤颗粒细胞病变，其中施万细胞起源可以排除或不明显。这些病变中有4例是以前未描述过的类型，与传统的皮肤颗粒细胞瘤不同，表现为息肉样结构、大量核分裂象、细胞异型性和原始免疫表型。我们建议将这些病变称为“原始息肉样颗粒细胞瘤”。其中1例发生在儿童，3例发生在成人。尽管有1例肿瘤局部复发，但截至目前，分别随访2年、4年、4年和16年，均无转移。可能需要更多病例和更长时间的随访来排除原始息肉样颗粒细胞瘤是低级别恶性肿瘤的可能性。另外2例颗粒细胞病变代表平滑肌肉瘤的变异型，其中1例发生广泛转移。最后1例是结节性基底细胞癌的颗粒细胞型。皮肤颗粒细胞瘤可表现出不同的分化程度和行为。病理学家不应将皮肤肿瘤中出现的细胞质颗粒等同于颗粒细胞型施万细胞瘤的诊断。
