Mucosal melanoma is rare and the occurrence of an invasive malignant melanoma metastasis 11 years post-initial diagnosis is equally uncommon. This is a case of a 66-year-old woman with a history of bilateral vulvar invasive melanoma, who presented with an enlarging inguinal mass with associated tenderness upon palpation. After a right inguinal excisional lymph node biopsy, the pathological findings determined the final diagnosis as metastatic melanoma. To the best of our knowledge, this is the first report of vulvar mucosal melanoma metastasis greater than 10 years after initial diagnosis and treatment in the English language. This case discusses how treatment options for metastatic mucosal melanoma pose a challenge in such cases where follow-up for medical care is lacking. It also highlights the need for further preventative techniques and research directed towards screening techniques, staging guidelines, and treatment options for mucosal melanoma.

BACKGROUND: PEComa is a mesenchymal tumor that can occur in various organs including the uterus and soft tissues. PEComas are composed of perivascular epithelioid cells, and angiomyolipoma (AML), clear cell sugar tumor (CCST), and lymphangiomyomatosis (LAM) are considered lesions of the same lineage as tumors of the PEComa family. Histologically, a common PEComa shows solid or sheet-like proliferation of epithelioid cells. This is accompanied by an increase in the number of dilated blood vessels. Here, we report a case of pancreatic PEComa with marked inflammatory cell infiltration.
METHODS: A 74-year-old male patient underwent an appendectomy for acute appendicitis. Postoperative computed tomography and magnetic resonance imaging revealed a 30 × 25 mm non-contrast-enhanced circular lesion in the tail of the pancreas. The imaging findings were consistent with a malignant tumor, and distal pancreatectomy was performed. Histologically, most area of the lesion was infiltrated with inflammatory cells. A few epithelioid cells with large, round nuclei, distinct nucleoli, and eosinophilic granular cytoplasm were observed. Spindle-shaped tumor cells were observed. Delicate and dilated blood vessels were observed around the tumor cells. Immunohistochemically, the atypical cells were positive for αSMA, Melan A, HMB-45, and TFE3. The cytological characteristics of the tumor cells and the results of immunohistochemical staining led to a diagnosis of pancreatic PEComa.
CONCLUSIONS: A histological variant known as the inflammatory subtype has been defined for hepatic AML. A small number of tumor cells present with marked inflammatory cell infiltration, accounting for more than half of the lesions, and an inflammatory myofibroblastic tumor-like appearance. To our knowledge, this is the first report of pancreatic PEComa with severe inflammation. PEComa is also a generic term for tumors derived from perivascular epithelioid cells, such as AML, CCST, and LAM. Thus, this case is considered an inflammatory subtype of PEComa. It has a distinctive morphology that is not typical of PEComa. This histological phenotype should be widely recognized.

Introduction: Lentigo maligna (LM) and lentigo maligna melanoma (LMM) predominantly affect the head and neck areas in elderly patients, presenting as challenging ill-defined pigmented lesions with indistinct borders. Surgical margin determination for complete removal remains intricate due to these characteristics. Morphological examination of surgical margins is the key form of determining successful treatment in LM/LMM and underpin the greater margin control provided through the Slow Mohs micrographic surgery (SMMS) approach. Recent assessments have explored the use of immunohistochemistry (IHC) markers, such as Preferentially Expressed Antigen in Melanoma (PRAME), to aid in LM/LMM and margin evaluation, leveraging the selectivity of PRAME labelling in malignant melanocytic neoplasms. Methods: A Novel double-labelling (DL) method incorporating both PRAME and MelanA IHC was employed to further maximise the clinical applicability of PRAME in the assessment of LM/LMM in SMMS biopsies. The evaluation involved 51 samples, comparing the results of the novel DL with respective single-labelling (SL) IHC slides. Results: The findings demonstrated a significant agreement of 96.1% between the DL method and SL slides across the tested samples. The benchmark PRAME SL exhibited a sensitivity of 91.3% in the SMMS specimens and 67.9% in histologically confirmed positive margins. Discussion: This study highlights the utility of PRAME IHC and by extension PRAME DL as an adjunctive tool in the assessment of melanocytic tumours within staged excision margins in SMMS samples.

Medullary thyroid cancer (MTC) represents a small proportion of thyroid cancers. In MTC, melanin production is extremely uncommon. Few case reports have documented this rare variant, and follow-up on these cases has been very limited. Our case studies a 51-year-old female who initially presented with goiter. This tumor recurred multiple times despite surgery with rapid growth and poor response to radiotherapy. Microscopic examination showed high-grade malignant neoplasm with lymphocytic differentiation. Immunohistochemical studies were diffusely positive for S100, SOX10, and Melan-A. Histology confirmed melanocytic medullary carcinoma that had undergone a high-grade transformation with loss of epithelial and neuroendocrine expression. Due to the scarcity and rarity of this subtype, further evaluation and case studies are needed for further categorization and prognostication.

BACKGROUND: Melanoblasts originate in the neural crest from where they migrate to peripheral tissues and differentiate into melanocytes. Alteration during melanocyte development and life can cause different diseases, ranging from pigmentary disorders and decreased visual and auditory functions, to tumours such as melanoma. Location and phenotypical features of melanocytes have been characterised in different species, yet data on dogs are lacking.
OBJECTIVE: This study investigates the expression of melanocytic markers Melan A, PNL2, TRP1, TRP2, SOX-10 and MITF in melanocytes of selected cutaneous and mucosal surfaces of dogs.
METHODS: At necropsy, samples from five dogs were harvested from oral mucosa, mucocutaneous junction, eyelid, nose and haired skin (abdomen, back, pinna, head).
METHODS: Immunohistochemical and immunofluorescence analyses were performed to assess marker expression.
RESULTS: Results showed variable expression of melanocytic markers in different anatomical sites, particularly within epidermis of haired skin and dermal melanocytes. Melan A and SOX-10 were the most specific and sensitive melanocytic markers. PNL2 was less sensitive, while TRP1 and TRP2 were seldomly expressed by intraepidermal melanocytes in haired skin. MITF had a good sensitivity, yet the expression often was weak.
CONCLUSIONS: Our results indicate a variable expression of melanocytic markers in different sites, suggesting the presence of subpopulations of melanocytes. These preliminary results pave the way to understanding the pathogenetic mechanisms involved in degenerative melanocytic disorders and melanoma. Furthermore, the possible different expression of melanocyte markers in different anatomical sites could influence their sensitivity and specificity when used for diagnostic purposes.
BACKGROUND: Les mélanoblastes proviennent de la crête neurale, d\'où ils migrent vers les tissus périphériques et se différencient en mélanocytes. L\'altération des ménanocytes durant leur développement et leur vie peut induire différentes maladies : des troubles pigmentaires et des fonctions visuelles et auditives, ainsi que tumeurs telles que le mélanome. La localisation et les caractéristiques phénotypiques des mélanocytes ont été précisées dans différentes espèces, mais ces données manquent chez le chien.
OBJECTIVE: L\'expression des marqueurs mélanocytaires Melan A, PNL2, TRP1, TRP2, SOX-10 et MITF est étudiée dans des échantillons de peau et de muqueuses de chiens. MATÉRIELS ET MÉTHODES: Durant l\'autopsie de cinq chiens, des échantillons de muqueuse buccale, de jonction cutanéo-muqueuse, de paupière, de nez et de peau velue (abdomen, dos, pavillon, tête) ont été prélevés ; des analyses immunohistochimiques et d\'immunofluorescence ont été réalisées pour évaluer l\'expression des différents marqueurs. RÉSULTATS: Les marqueurs mélanocytaires sont exprimés de façon variable selon les différents sites anatomiques, en particulier au sein de l\'épiderme de la peau velue et des mélanocytes dermiques. Melan A et SOX-10 sont les marqueurs mélanocytaires les plus spécifiques et les plus sensibles. PNL2 apparait moins sensible, tandis que TRP1 et TRP2 sont rarement exprimés par les mélanocytes intraépidermiques de la peau velue. MITF avait une bonne sensibilité, mais l\'expression était souvent faible.
UNASSIGNED: Les marqueurs mélanocytaires sont exprimés de façon variable dans différents sites anatomiques, suggérant la présence de sous-populations mélanocytaires. Ces résultats préliminaires ouvrent la voie à la compréhension des mécanismes pathogéniques impliqués dans les maladies mélanocytaires dégénératives et le mélanome. De plus, ces éventuelles différences d’expression des marqueurs mélanocytaires selon le site anatomique pourraient influencer la sensibilité et la spécificité des analyses utilisant ces marqueurs à visée diagnostique.
INTRODUCCIÓN: los melanoblastos se originan en la cresta neural desde donde migran a los tejidos periféricos y se diferencian en melanocitos. La alteración durante el desarrollo y la vida de los melanocitos puede causar diferentes enfermedades, que van desde trastornos pigmentarios y disminución de las funciones visuales y auditivas, hasta tumores como el melanoma. La ubicación y las características fenotípicas de los melanocitos se han caracterizado en diferentes especies, pero faltan datos sobre perros. OBJETIVO: Este estudio investiga la expresión de los marcadores melanocíticos Melan A, PNL2, TRP1, TRP2, SOX-10 y MITF en melanocitos de superficies cutáneas y mucosas seleccionadas de perros. MATERIALES Y MÉTODOS: durante las necropsias, se obtuvieron muestras de cinco perros de mucosa oral, unión mucocutánea, párpado, nariz y piel con pelo (abdomen, espalda, pabellón auricular, cabeza); Se realizaron análisis inmunohistoquímicos y de inmunofluorescencia para evaluar la expresión de los marcadores. RESULTADOS: Los resultados mostraron una expresión variable de marcadores melanocíticos en diferentes sitios anatómicos, particularmente en la epidermis de la piel con pelos y en melanocitos dérmicos. Melan A y SOX-10 fueron los marcadores melanocíticos más específicos y sensibles. PNL2 fue menos sensible, mientras que TRP1 y TRP2 rara vez se expresaron en melanocitos intraepidérmicos en la piel con cabello. MITF tenía una buena sensibilidad, pero la expresión a menudo era débil. CONCLUSIONES Y RELEVANCIA CLÍNICA: Nuestros resultados indican una expresión variable de marcadores melanocíticos en diferentes sitios, lo que sugiere la presencia de subpoblaciones de melanocitos. Estos resultados preliminares inician el camino para comprender los mecanismos patogénicos implicados en los trastornos melanocíticos degenerativos y el melanoma. Además, la posible expresión diferente de los marcadores de melanocitos en diferentes sitios anatómicos podría influir en su sensibilidad y especificidad cuando se utilizan con fines de diagnóstico.
UNASSIGNED: Melanoblasten stammen aus der Neuralleiste, von der sie ins periphere Gewebe auswandern und zu Melanozyten ausdifferenzieren. Veränderungen während der Entwicklung der Melanozyten und ihres Lebenslaufs können verschiedene Krankheiten bedingen, von Pigmentunregelmäßigkeiten und verminderten visuellen und auditorischen Funktionen bis hin zu Tumoren wie Melanome. Die Lokalisation und die phänotypischen Merkmale wurden bei verschiedenen Spezies beschrieben, beim Hund gibt es jedoch bisher keine Daten. ZIEL: Diese Studie untersucht die Exprimierung von Melanozytenmarkern Melan A, PNL2, TRP1, TRP2, SOX-10 und MITF an Melanozyten von selektiven kutanen und mucokutanen Oberflächen von Hunden.
UNASSIGNED: Bei der Nekropsie wurden Proben aus der oralen Mukosa, mucokutanen Übergängen, Augenlid, Nase und behaarter Haut (Bauch, Rücken, Pinna, Kopf) von fünf Hunden genommen; immunhistochemische und Immunfluoreszenz-Analyse wurde durchgeführt, um eine Markerexprimierung zu erfassen.
UNASSIGNED: Die Ergebnisse zeigten eine unterschiedliche Exprimierung der Melanozytenmarker an verschiedenen anatomischen Stellen, vor allem innerhalb der Epidermis der behaarten Haut und der dermalen Melanozyten. Melan A und SOX-10 waren die spezifischsten und sensitivsten Melanozytenmarker. PNL2 waren weniger sensitiv, während TRP1 und TRP2 selten an intraepidermalen Melanozyten der behaarten Haut exprimiert wurden. MITF zeigte eine gute Sensibilität, obwohl die Exprimierung oft nur schwach war. SCHLUSSFOLGERUNGEN UND KLINISCHE RELEVANZ: unsere Ergebnisse zeigten eine variable Exprimierung der Melanozytenmarker an verschiedenen Stellen, was auf das Auftreten unterschiedlicher Subpopulationen der Melanozyten hinweist. Diese vorläufigen Ergebnisse ebnen den Weg für ein Verständnis der pathogenen Mechanismen, die bei degenerativen Melanozyten-Veränderungen und beim Melanom eine Rolle spielen. Weiters könnte die möglicherweise unterschiedliche Exprimierung der Melanozytenmarker an verschiedenen anatomischen Stellen ihre Sensibilität und Spezifität beeinflussen, wenn sie für diagnostische Zwecke eingesetzt werden.
背景: メラノブラストは神経堤で発生し、そこから末梢組織へ移動してメラノサイトに分化する。メラノサイトの発生・生育過程における変化は、色素障害、視覚・聴覚機能の低下からメラノーマなどの腫瘍に至るまで、様々な疾患の原因となる可能性がある。メラノサイトの位置や表現型は、様々な動物種で明らかにされているが、犬に関するデータは不足している。 目的: 本研究の目的は、犬の皮膚・粘膜のメラノサイトにおけるメラノサイトマーカーMelan A, PNL2, TRP1, TRP2, SOX-10, MITFの発現を検討することであった。 材料と方法: 剖検時に、5頭の犬から口腔粘膜、粘膜皮膚接合部、眼瞼、鼻および有毛皮膚(腹部、背部、耳介、頭部)のサンプルを採取し、免疫組織化学および免疫蛍光解析を行い、マーカーの発現を評価した。 結果 その結果、解剖学的な部位によってメラノサイトマーカーの発現が異なり、特に有毛皮膚表皮内および真皮のメラノサイトの発現にばらつきがあることが判明した。Melan AおよびSOX-10は、最も特異的で感度の高いメラノサイトマーカーであった。PNL2は感度が低く、TRP1およびTRP2は有毛皮膚内の表皮内メラノサイトでほとんど発現していなかった。MITFは感度が高いが、発現が弱い場合が多い。 結論と臨床的意義: 今回の結果は、部位によってメラノサイトマーカーの発現が異なることを示し、メラノサイトの亜集団の存在を示唆するものであった。これらの予備的な結果は、変性メラノサイト障害やメラノーマに関わる発症メカニズムの理解に道を開くものである。さらに、解剖学的部位によってメラノサイトマーカーの発現が異なる可能性があることから、診断目的で使用する場合には、その感度や特異性に影響を与える可能性がある。.
背景: 黑素细胞起源于神经嵴，从那里迁移到周围组织并分化为黑素细胞。黑素细胞发育和生活过程中的变化会导致不同的疾病，从色素性疾病、视觉和听觉功能下降，到黑色素瘤等肿瘤。黑色素细胞的位置和表型特征已经在不同的物种中得到了表征，但关于犬的数据仍然缺乏。 目的: 本研究研究黑素细胞标志物黑素A、PNL2、TRP1、TRP2、SOX-10和MITF在犬皮肤和粘膜表面黑素细胞中的表达。 材料和方法 尸检时，从五只犬的口腔粘膜、粘膜皮肤连接处、眼睑、鼻子和毛发皮肤(腹部、背部、耳廓、头部)采集样本；进行免疫组织化学和免疫荧光分析以评估标记物的表达。 结果: 结果显示黑素细胞标记物在不同解剖部位的表达不同，尤其是在毛发皮肤的表皮和真皮黑素细胞内。黑素A和SOX-10是最特异和敏感的黑素细胞标志物。PNL2较不敏感，而TRP1和TRP2仅在毛发皮肤表皮内黑素细胞中表达。MITF具有良好的敏感性，但表达往往较弱。 结论和临床相关性: 我们的结果表明，不同部位的黑素细胞标志物表达不同，表明存在黑素细胞亚群。这些初步结果为理解退行性黑素细胞疾病和黑色素瘤的发病机制铺平了道路。此外，当用于诊断目的时，黑素细胞标记物在不同解剖部位的可能不同表达可能会影响其敏感性和特异性。.
UNASSIGNED: Os melanoblastos se originam da crista neural, de onde eles migram para os tecidos periféricos e se diferenciam em melanócitos. Alterações durante o desenvolvimento e ao longo da vida dos melanócitos pode causar diferentes doenças, desde alterações pigmentares e redução das funções auditivas e visuais, a tumores como o melanoma. Localização e características fenotípicas dos melanócitos têm sido detalhadas em várias espécies, mas em cães os dados ainda são escassos.
OBJECTIVE: Este estudo investiga a expressão dos marcadores melanocíticos Melan A, PNL2, TRP1, TRP2, SOX-10 e MITF nos melanócitos de determinadas superfícies cutâneas e mucosas em cães. MATERIAIS E MÉTODOS: À necrópsia, foram coletadas amostras de mucosa oral, junção mucocutânea, pálpebra, nariz e pele pilosa (abdômen, dorso, orelha, cabeça); para se avaliar a expressão dos marcadores, realizou-se imuno-histoquímica e imunofluorescência.
RESULTS: Os resultados demonstraram expressão de marcadores melanocíticos em diferentes sítios anatômicos, particularmente na epiderme da pele pilosa e nos melanócitos dérmicos. Melan A e SOX-10 foram os marcadores melanocíticos mais específicos e sensíveis. PNL2 foi menos sensível, enquanto TRP1 e TRP2 foram raramente expressados por melanócitos intraepidérmicos na pele pilosa. MITF apresentou boa sensibilidade, apesar de a sua expressão ser frequentemente fraca. CONCLUSÕES E RELEVÂNCIA CLÍNICA: Nossos resultados indicam uma expressão variável de marcadores melanocíticos em diferentes regiões, sugerindo a presença de subpopulações de melanócitos. Estes resultados preliminares embasam a compreensão dos mecanismos patogenéticos envolvidos nas doenças melanocíticas degenerativas e no melanoma. Além disso, a possível diferença na expressão de marcadores melanocíticos em diferentes sítios anatômicos pode influenciar a sua sensibilidade e especificidade quando utilizado para diagnóstico.

OBJECTIVE: Divided nevus with verrucous hyperplasia will always recur after surgery but non-verrucous divided eyelid nevus rarely recur. This study analyzed the differential expression of Ki-67, S100, Melan A and HMB45 and identified the correlation between the clinical and histopathological features of verrucous and non-verrucous divided eyelid nevus.
METHODS: This study included 29 patients, of whom 8 patients had verrucous divided nevus. Immunohistochemistry labeling was used to assess the expression of Ki-67, S100, Melan A and HMB45 after excision. The difference between verrucous and non-verrucous divided eyelid nevus was analyzed.
RESULTS: The patients\' ages ranged from 2 to 59 years, with a mean age of 19 years. The lesion size ranged from 1.5 to 2.0 cm in diameter and invaded the eyelid margins and the posterior lamella of the eyelids. Immunohistochemistry labeling showed strong positivity for approximately 98.5% of S100 and positive staining for approximately 27.6% of Ki-67, 72.4% of Melan A and 6.8% of HMB45. However, Ki-67 was significantly upregulated in verrucous divided nevus of the eyelids compared with non-verrucous divided nevus, with approximately 38.8% upregulation in verrucous and 18.3% upregulation in non-verrucous nevus.
CONCLUSIONS: This study correlated the clinic-pathologic features of verrucous divided eyelid nevus by means of statistically analyzing the varied clinical features and pathological impressions. The significant over-expression of S100 may be used as an indicator of divided nevus of the eyelids, and the over-expressed Ki-67 may contribute to the recurrence of verrucous divided nevus. High expression of HMB45 and Melan A may represent malignant transformation.

Adrenocortical cancer (ACC) is a rare endocrine malignancy of the adrenal cortex, which has an unfavorable prognosis and extremely aggressive clinical behavior in most cases. Nevertheless, cases of a more favorable disease course with late metastasis and slow progression have been described. In 2017, the International Agency for Research on Cancer (IARC) and the World Health Organization (WHO) in the 4th edition of the Classification of Tumors of the Endocrine Organs identified histological variants of ACC, such as classical, oncocytic, myxoid, and sarcomatoid ones, indicating the morphological heterogeneity of this tumor.
OBJECTIVE: To provide a detailed description of the morphological variants of ACC with an emphasis on their histological characteristics and the expression of immunohistochemical markers.
METHODS: A total of 75 cases of ACC were analyzed in the adult population diagnosed as having the morphological variants in accordance with the International Histological Classification of Adrenal Tumors (WHO, 2017). Monoclonal antibodies to SF1, Inhibin A, Melan A, Ki-67, p53, and antimitochondrial antibodies were used for immunohistochemical diagnosis.
RESULTS: The classic, oncocytic, and myxoid subtypes of ACC were found in 51 (68%), 15 (20%), and 9 (12%) cases, respectively. The functional activity of the tumors was observed in 43% (n=18) in the classic variant of ACC; moreover, the clinical picture was manifested by the symptoms of hypercorticism (38%) and virilization (5%). There were no significant differences in hormonal activity between different morphological variants. The characteristics of the above histological variants of the tumor was determined with a description of growth patterns that can improve the diagnosis of ACC. The diagnosis of ACC can be confirmed by an immunohistochemical study; the required minimum panel of markers should include SF1, Melan A, and Inhibin A. The Ki-67 proliferative activity index showed significant differences (p=0.0056) when it was determined in the morphological variants of ACC.
CONCLUSIONS: Despite the determination of a minimal immunohistochemical panel to confirm the diagnosis of ACC, it is important to remember that each histological variant may be characterized by the different expression of immunohistochemical markers. The identification of morphological variants of ACC and the use of specific, sensitive, and prognostically significant immunohistochemical markers will allow clinicians and pathologists to more accurately judge the biological properties of this tumor and the clinical course of the disease.
Адренокортикальный рак (АКР) представляет собой редкую злокачественную эндокринную опухоль коры надпочечников, в большинстве случаев имеющую неблагоприятный прогноз и крайне агрессивное клиническое поведение. Тем не менее описаны случаи более благоприятного течения заболевания с поздним метастазированием и медленным прогрессированием. В 2017 г. Международным агентством по изучению рака (International Agency for Research on Cancer, IARC) и Всемирной организацией здравоохранения (ВОЗ) в 4-м издании Классификации опухолей эндокринных органов (WHO Classification of Tumors Pathology and Genetics) выделены гистологические варианты АКР: классический, онкоцитарный, миксоидный и саркоматоидный, свидетельствующие о морфологической неоднородности такой опухоли.
UNASSIGNED: Подробное описание морфологических вариантов АКР с акцентом на их гистологических характеристиках и экспрессии иммуногистохимических маркеров.
UNASSIGNED: В настоящем исследовании выполнен анализ 75 случаев АКР во взрослой популяции с диагностикой морфологических вариантов в соответствии с Международной гистологической классификацией опухолей надпочечника (ВОЗ, 2017 г.). Для иммуногистохимической диагностики использованы моноклональные антитела к SF1, Inhibin A, Melan A, Ki-67, р53, антимитохондриальные антитела.
UNASSIGNED: Классический подтип АКР выявлен у 51 (68%) человека, онкоцитарный — у 15 (20%), миксоидный — у 9 (12%). Функциональная активность опухолей наблюдалась в 43% (18 случаев) при классическом варианте АКР, при этом наиболее часто клиническая картина проявлялась симптомами гиперкортицизма (38%) и вирилизации (5%). Достоверных различий в гормональной активности между различными морфологическими вариантами не обнаружено. Определена характеристика вышеописанных гистологических вариантов опухоли с описанием паттернов роста, которые позволяют улучшить диагностику АКР. Подтверждение диагноза АКР возможно только при проведении иммуногистохимического исследования, необходимая минимальная панель маркеров должна включать: SF1, Melan A, Inhibin A. Индекс пролиферативной активности Ki-67 показал достоверные различия (p=0,0056) при его определении в морфологических вариантах АКР.
UNASSIGNED: Несмотря на определение минимальной иммуногистохимической панели, позволяющей подтвердить диагноз АКР, важно помнить, что каждый гистологический вариант может характеризоваться различной выраженностью экспрессии иммуногистохимических маркеров. Выделение морфологических вариантов АКР и использование специфичных, чувствительных и прогностически значимых иммуногистохимических маркеров позволят клиницистам и патоморфологам более точно судить о биологических свойствах этой опухоли и клиническом течении заболевания.

Background: To present basic demographic and clinical characteristics of patients with adrenocortical carcinoma (ACC), to determine the overall survival rate and to analyze the results of immunohistochemical staining and its correlation with the length of survival.Material and methods: The study was conducted during the period between 1996 and 2010 and included 30 patients with ACC. Immunohistochemical staining (MMP9, melan A, inhibin, caltretinin, D2-40, synaptophysin and Ki-67) was performed.Results: ACC was diagnosed in 19 females and 11 men (1.7:1). The average age was 50.1 years. The median tumor size was 10 cm, the median weight 400 g. Majority of subjects had positive immunohistochemical staining for the markers of interest. Patients with any negative staining had shorter cancer-specific survival than ones with positive staining. According to the log-rank test results as well as according to the results of the univariate Cox analysis, negative staining for inhibin, D2-40 and synaptophysin and Ki-67 expression ≥7% were associated with poorer prognosis.Conclusions: The results of our study suggest that the absence of staining for some immunohistochemical markers and increased expression of Ki-67 are associated with a poorer prognosis and shorter survival of patients with ACC. Immunohistochemical markers may serve as a prognostic factor for ACC.

Hepatic perivascular epithelioid cell tumors (PEComas) are very rare. We report a primary hepatic PEComa with a review of the literature. A 56-year-old women presented with a nodular mass detected during the management of chronic renal failure and chronic hepatitis C. Diagnostic imaging studies suggested a nodular hepatocellular carcinoma in segment 5 of the liver. The patient underwent partial hepatectomy. A brown-colored expansile mass measuring 3.2×3.0 cm was relatively demarcated from the surrounding liver parenchyma. The tumor was mainly composed of epithelioid cells that were arranged in a trabecular growth pattern. Adipose tissue and thick-walled blood vessels were minimally identified. A small amount of extramedullary hematopoiesis was observed in the sinusoidal spaces between tumor cells. Tumor cells were diffusely immunoreactive for human melanoma black 45 (HMB45) and Melan A, focally immunoreactive for smooth muscle actin, but not for hepatocyte specific antigen (HSA).

Angiomyolipoma with epithelial cysts (AMLEC) is a distinctive variant of angiomyolipoma characterized by grossly apparent epithelial cysts and a cellular, müllerian-like subepithelial stroma. Some authors suspect that the epithelial cysts mainly represent dilated entrapped native renal collecting duct epithelium, while other authors think that they represented true epithelial differentiation of the AML. Recently, it has been reported that obvious immunolabeling of melanocytic markers such as Melan A and HMB45, which are often immunolabeled in classical angiomyolipoma, are present in epithelial cysts in cases of AMLEC. Here, we report the case of a 43-year-old Japanese woman with AMLEC, and attempt to elucidate the significance of melanocytic marker immunolabeling in the cyst\'s epithelium. In the present case, Melan A was labeled in the cyst\'s epithelium, and was thought to reflect its labeling in renal tubules existing in the renal parenchyma outside the tumor. This finding may indicate that the cyst epithelium is derived from entrapped renal tubules inside the AML. Non-immunolabeling of the estrogen and progesterone receptors in the cyst epithelium may also suggest that the cyst epithelium is not neoplastic, in contrast to their labeling in neoplastic cells existing in cyst wall. Further examination, such as molecular analysis, is needed to determine whether these epithelial cysts is neoplastic or non-neoplastic.