面部色素痣的外科治疗

面部色素痣较常见于鼻、颊、额、颧区，部分病变累及一侧面部的多个区域。这些病变手术切除后的缺损主要采用全厚皮片移植、直接拉拢缝合或邻近皮瓣整复。     

原发性色素性结节状肾上腺皮质病(附4例报告)

目的：探讨原发性色素性结节状肾上腺皮质病(PPNAD)的临床表现和诊断治疗方法。方法：总结4例PPNAD的临床资料，4例均有库欣综合征的临床表现，内分泌检查结果提示为功能自主性肾上腺皮质肿瘤，但影像学检查并未发现肾上腺肿瘤。结果：4例患者均行单侧肾上腺全切除术，手术标本均表现为肾上腺大小正常或轻度增大，外表和切面上见黑色或深褐色小结节，结节间皮质萎缩，光镜下见组成结节的细胞体积大，脑质嗜伊红染色、颗粒状，部分细胞脑质中的颗粒状色素颗粒具脂褐质染色特征。结论：PPNAD在青少年中是一种引起库欣综合征的罕见病因，双侧肾上腺切除术是治愈本病的方法。     

Relative frequency of solitary melanocytic lesions of the oral mucosa

Background: Solitary pigmented lesions of melanocytic origin are uncommon in the oral mucosa. These lesions include the oral and labial melanotic macule, oral melanocytic nevus, oral melanoacanthoma, oral melanoma and atypical melanocytic proliferation. The purpose of the study was twofold: to report a large series of solitary melanocytic lesions from one source, and to determine the relative frequency of these lesions. Methods: The study was based on a systematic search of the files of the Pacific Oral and Maxillofacial Pathology Laboratory, University of the Pacific, San Francisco for solitary pigmented melanocytic lesions (benign and malignant) accessed during the years 1984–2002. Results: Of the 89 430 biopsies accessed during the 19‐year period, 773 (0.83%) cases of solitary pigmented melanocytic lesions in the oral mucosa were identified. Oral and labial melanotic macules were the most common melanocytic lesions comprising 86.1% of the entire group and 0.7% of the total number of accessed biopsies. The vermilion border and gingiva were the most common sites (31.1% and 31.0% respectively). Oral melanocytic nevi comprised 11.8% of the entire melanocytic group and 0.1% of the total number of biopsies. The most common site was the palate (44%). Intramucosal nevi were the most common (64%), followed by compound nevi (16.5%) and common blue nevi (16.5%). Junctional nevi were uncommon (3.0%). Oral melanoacanthoma comprised only 0.9% of the entire melanocytic group and 0.008% of the total number of biopsies. Oral melanoma and atypical melanocytic proliferation were the least common lesions each comprising 0.6% of the entire melanocytic group and 0.006% of the total number of biopsies. The most common site for oral melanoma was the palate (60%). Conclusion: The palate was the most common location for both melanocytic nevi and oral melanoma. Thus, all melanocytic lesions in the palate should be viewed with caution and biopsy is recommended to rule out melanoma. Further studies are required to elucidate the entity of oral atypical melanocytic proliferation.     

牙源性脓肿标本中的产黑色素类杆菌群菌株分离鉴定

本文对４４例牙源性脓肿（尖周脓肿２４例，牙周脓肿１２例，冠周脓肿８例）脓液标本中的产黑色素类杆菌群菌株进行分离、培养、鉴定。其中３８例存在产黑菌群，以中间型类杆菌阳性率最高。牙龈类杆菌在牙周脓肿中检出率最高。２４例尖周脓肿可分离出６株牙髓类杆菌而其它两种牙源性脓肿则无牙髓类杆菌，推测该菌与牙髓尖周感染的病理过程有关。     

Myelolipoma: A New Adrenal Finding in Carney's Complex?

Pigmented nodular cortical hyperplasia, a rare cause of Cushing’s syndrome, is characterized by resistance to inhibition with dexamethasone and normal sized adrenal glands with multiple, small pigmented nodules. The disorder may be a component of a syndrome inherited as an autosomal dominant pattern that includes intra- and extracardiac myxomas, lentiginous lesions, blue nevi, other functional endocrine tumors, and peripheral nerve tumors (Carney’s complex). We report a patient in whom bilateral myelolipomas were found, in addition to the usual features of this complex. A 29-yr-old man was admitted to the hospital for Cushing’s syndrome of probably more than 15 yr duration. Physical examination showed diffuse facial hyperchromatic macules, 0.2–0.5 cm, predominantly around the lips and on the palmar surfaces of the fingers. Results with dexamethasone suppression nocturnal testing (1 and 8 mg) were compatible with an adrenal adenoma. The computed tomography (CT) of the sella turcica was normal. Adrenal CT showed a tumor in the left gland with a double component: one solid and another suggestive of fat, consistent with an angiomyelolipoma. Following 5 wk treatment with ketoconazole, 800 mg per day po, serum cortisol decreased to 5.9 μg/dL, morning and evening, respectively. Bilateral adrenalectomy was performed. Pathologic examination revealed pigmented nodular cortical hypersplasia and a dominant myelolipoma in the left adrenal. A microscopic myelolipoma was identified in the right adrenal. An echocardiogram showed a mass on the posterior wall of the left ventricle which was a myxoma. Study of the patient's family disclosed two sisters with facial lentigines. Echocardiograms were performed on all available first degree relatives: all were normal. Nocturnal inhibition with dexamethasone revealed that one of the patient’s sisters with lentigines also had hypercortisolism. Myelolipoma has been reported in association to Cushing syndrome in humans and experimentally after pituitary extracts in animals. The relationship between this finding and the Carney’s complex remain elusive.     

Malignant schwannoma with melanocytic and neuroepithelial differentiation in an infant with congenital giant melanocytic nevus: A complex neurocristopathy

We describe an infant girl, born with a pigmented giant nevus, who developed a malignant schwannoma in the retroperitoneum at 16 months of age. At birth the nevus covered over 50% of her body and histologically was a compound nevus with extension into the deep dermis surrounding dermal appendages. The malignant schwannoma was biphasic with areas composed of spindle and round cells. Ultrastructurally, the majority of the tumor cells exhibited a Schwann cell phenotype, but neuroepithelial and melanocytic cells were identified as well. We believe that this constellation of findings represents a form of neurocristopathy. Neurocristopathy, as defined by Bolande (Hum Pathol 5:409–429, 1974), is a disease that results from aberrations in the migration, growth, or cytodifferentiation of neural crest tissues. These diseases may be simple (a singular pathologic process, usually localized) or complex (multiple neuroectodermal lesions). We report this case because the occurrence of retroperitoneal malignant schwannoma arising in a 16-month-old infant born with a pigmented giant nevus is unique, and may represent a previously undescribed form of a complex neurocristopathy.     

色素绒毛结节性滑膜炎28例报道

目的探讨色素绒毛结节性滑膜炎(PVS)的临床病理特点、治疗和预后的关系。方法本组PVS28例,男13例,女15例,年龄12～53岁。膝关节15例,手指7例,足背4例,颞颌及骶髂关节各1例,多肢体1例。局限型19例,弥漫型9例。伴外伤史3例,骨破坏4例,恶变2例,半月板破裂1例,滑膜血管瘤1例。均行滑膜切除,早期操练,3例加放疗。28例均经病理证实。结果22例随访2～18年,13例功能良好;9例复发5例功能欠佳,2例截指(趾),2例死亡。放疗3例均复发。结论PVS临床病理均有肿瘤的特征,可恶变和转移,易复发,最佳治疗是彻底的滑膜切除而不是放疗。     

Folliculotropism in pigmented facial macules: Differential diagnosis with reflectance confocal microscopy

Pigmented facial macules are common on sun damage skin. The diagnosis of early stage lentigo maligna (LM) and lentigo maligna melanoma (LMM) is challenging. Reflectance confocal microscopy (RCM) has been proven to increase diagnostic accuracy of facial lesions. A total of 154 pigmented facial macules, retrospectively collected, were evaluated for the presence of already‐described RCM features and new parameters depicting aspects of the follicle. Melanocytic nests, roundish pagetoid cells, follicular infiltration, bulgings from the follicles and many bright dendrites and infiltration of the hair follicle (ie, folliculotropism) were found to be indicative of LM/LMM compared to non‐melanocytic skin neoplasms (NMSNs), with an overall sensitivity of 96% and specificity of 83%. Concerning NMSNs, solar lentigo and lichen planus‐like keratosis resulted better distinguishable from LM/LMM because usually lacking malignant features and presenting characteristic diagnostic parameters, such as epidermal cobblestone pattern and polycyclic papillary contours. On the other hand, distinction of pigmented actinic keratosis (PAK) resulted more difficult, and needing evaluation of hair follicle infiltration and bulging structures, due to the frequent observation of few bright dendrites in the epidermis, but predominantly not infiltrating the hair follicle (estimated specificity for PAK 53%). A detailed evaluation of the components of the folliculotropism may help to improve the diagnostic accuracy. The classification of the type, distribution and amount of cells, and the presence of bulging around the follicles seem to represent important tools for the differentiation between PAK and LM/LMM at RCM analysis.     

Combined melanocytic and sweat gland neoplasm: cell subsets harbor an identical HRAS mutation in phacomatosis pigmentokeratotica

Phacomatosis pigmentokeratotica (PPK) is characterized by the co‐existence of epidermal nevi and large segmental speckled lentiginous nevi of the papulosa type. PPK, previously explained as ‘twin spot’ mosaicism due to the postzygotic crossing‐over of two homozygous recessive mutations, has recently been shown to derive from one postzygotic activating RAS mutation. Epidermal nevi, including those in PPK, are known to give rise to neoplasms such as trichoblastoma and basal cell carcinoma. Within speckled lentiginous nevi, Spitz nevi and melanoma have been well documented. We report a case of PPK with a combined melanocytic and adnexal neoplasm presenting where the nevi conjoined. Using next‐generation sequencing techniques, we were able to identify the same HRAS G13R mutation within both components of the tumor, and to show the absence of additional mutated modifier genes in a panel of 300 cancer‐related genes. Given the genetic findings in this rare tumor‐type, we suggest that this case may be used as a model for understanding the development of biphenotypic neoplasia or intratumoral heterogeneity in some cases.