Malignant glomus tumor of kidney: the first reported case and review of literature

Glomus tumors are mesenchymal neoplasms and are rarely seen in visceral organs like the kidney. Our patient presented with a metastatic, malignant, and highly aggressive glomus tumor in the kidney. In our extensive literature review, we did not come across even a single case of malignant glomus tumor arising in the kidney. We report the clinical presentation, radiologic, and pathological features of our case. Immunohistochemical findings that distinguish our case from other reported cases of glomus tumors arising from the kidney have been discussed. We have also reviewed the criteria for malignancy and other reported malignant glomus tumors.     

Pulmonary glomus tumor

Glomus tumor (GT) is an infrequent but distinct neoplasm. Pulmonary GT is a rare neoplasm with only a few cases reported in the literature. These tumors are usually benign and, although rare, tumors with aggressive behavior have been reported. The tumor size, location, and histomorphological features may be useful in predicting tumor behavior. We present here a case of pulmonary GT that was initially diagnosed as a typical carcinoid tumor. The differential diagnosis as well as the recent classification of GTs is discussed along with a review of literature.     

Malignant granular cell tumor with unusual histological features

Malignant granular cell tumor, although uncommon, should be differentiated from a number of granular cell-containing tumors. Reported herein is a distinctive variant of malignant granular cell tumor, clinically presenting as a rapidly enlarging scrotal mass, in which some areas morphologically displayed features indistinguishable from Kaposi sarcoma. Cells in areas simulating Kaposi sarcoma were immunohistochemically the same as typical granular cells in other portions of the tumor. The recognition of this pattern is important because it may predominate and overshadow the original nature of tumor.     

Symplastic glomus tumor: report of a challenging lesion with literature review

Glomus tumors are uncommon mesenchymal tumors whose cells closely resemble those of a normal glomus body, and are found most commonly in the hand. Recently, a symplastic form exhibiting marked nuclear atypia, in the absence of any other malignant features, has been described. To date, only 14 cases of symplastic glomus tumor have been reported in the literature; hence, very little information is available about its diagnosis, treatment, and biological behavior. The case reported here concerns a symplastic glomus tumor occurring in the right index finger of a 62-year-old woman. We reviewed the literature concerning previously reported cases, placing emphasis on the documented biological behavior, treatment, and demographic trend. Physicians must be aware of this morphological variation of glomus tumor to avoid the mistake of malignancy, which results in over-treatment of the patient.     

Cytopathology approach to rare salivary gland lesions with oncocytic features

Salivary glands located in the head and neck area are known to have different lesions with prominent oncocytic features. Fine needle aspiration is usually the initial approach in the management of these lesions, whether they represent neoplastic or non‐neoplastic processes. Owing to the limited material present upon cytopathologic examination, knowledge of variants and subtypes of various oncocytic lesions that can occur in this area is of major importance to provide the most accurate interpretation to the patients and clinicians. In this article, we selected rare lesions of the salivary glands that may exhibit oncocytic changes, and provided a brief discussion of each one of them, with emphasis on challenges in navigating the differential diagnosis that these cases may pose, from the cytopathology standpoint.     

Malignant glomus tumor of the lung

Primary malignant glomus tumors of the lung are extremely rare, and to our knowledge, only three cases have been described to date. We report one such case in a 53-year-old man who presented with a persistent dry cough. Chest computed tomography scans demonstrated an irregularly shaped mass in the right lower lobe of the lung. Many small nodules were distributed from the main tumor to the periphery, along with bronchovascular bundles. Right lower lobectomy was performed under the diagnosis of lung tumor. The tumor was located in the proximal portion of the right lower lobe and extended along the pulmonary arteries. Histological examination revealed a sheet-like proliferation of epithelioid glomus cells and fascicles of spindle cells. The presence of increased mitotic activity, tumor necrosis and prominent intravascular invasion suggested malignancy. The tumor cells were immunoreactive for vimentin, calponin, h-caldesmon, and alpha-smooth muscle actin, which indicated definitive smooth muscle differentiation. We believe that this is the fourth reported case of malignant glomus tumor of the lung.     

结肠嗜酸细胞癌2例临床病理分析

目的 探讨结肠嗜酸细胞癌的病理组织学特点、诊断及鉴别诊断。方法 对2例结肠嗜酸细胞癌手术切除标本进行光镜、免疫组化染色及电镜观察。结果 结肠嗜酸细胞癌组织学特点为细胞圆形、多边形,胞质丰富,胞质内见弥漫分布的强嗜酸性颗粒,细胞核中度异型性,核仁明显;癌细胞呈巢状排列,伴有腺管及乳头形成。免疫表型：癌细胞CK19、EMA阳性表达,不表达SMA、S-100蛋白及CgA。PAS染色阴性。电镜观察细胞质充满大量线粒体,缺乏内分泌及外分泌颗粒。结论 嗜酸细胞癌是不同于低分化腺癌及神经内分泌癌的一种特殊类型细胞癌。     

Pancreatic serous microcystic adenoma with extensive oncocytic change

Herein is reported a case of pancreatic serous microcystic adenoma with extensive oncocytic change in a 73-year-old woman. Histologically the tumor consisted of numerous small cysts, separated by thin or broad fibrous septa. These cysts were lined with uniform cells having abundant eosinophilic granular cytoplasm, which was negatively or weakly stained with PAS. Immunohistochemically, the cyst-lining cells were positive for cytokeratin (CK) 7, CK19, MUC1, MUC6, α-inhibin, and neuron-specific enolase (NSE), and negative for CK8, CK20, MUC2, and MUC5AC; these immunoprofiles coincide with those of serous microcystic adenoma. Immunostaining with anti-mitochondrial antibody showed dense granular positivity in the cytoplasm, which suggested an oncocytic phenotype. Thus, this case is considered a variant of serous microcystic adenoma characterized by extensive oncocytic change. To the authors' knowledge no similar case has been reported in the literature. It may pose problems in the differential diagnosis of the cystic pancreatic tumors with oncocytic change, but can be diagnosed on histology and immunohistochemistry.     

Features of gastric glomus tumor: a clinicopathologic,immunohistochemical and molecular retrospective study

Glomus tumor (GT) of the stomach is a rare mesenchymal tumor. There have been few detailed studies on these tumors. A total of 1894 cases of resected gastric mesenchymal tumors were collected and eleven confirmed gastric GTs were studied. The clinical, pathological, immunohistochemical, ultrastructural and molecular characteristics of the tumors were analyzed through a retrospective study. Histologically, most tumors had gastric smooth muscle immediately adjacent and surrounding the tumor. Tumor cells around blood vessels were small, uniform, and round. Foci of hyaline and myxoid changes were observed. Prominent clear cell features were observed in two tumors. Positive expression of α-smooth muscle actin (α-SMA), laminin, collagen type IV, and vimentin was detected by immunohistochemical analysis in all patients. However, in clear cell areas the expression of α-SMA, laminin, and type IV collagen were mild, while Syn was positive. Moreover, myofibrils and neuroendocrine granules were also present in the cytoplasm of these cells. No C-kit or PDGFR-α genetic mutations were detected in all patients. To conclude, Our results show that GTs in the stomach are histologically and immunophenotypically fully comparable with the glomus tumors of peripheral soft tissues. Neuroendocrine granules and neuroendocrine differentiation were identified in some of the gastric GT cells. Thus, a novel subtype of gastric glomus tumor expressing neuroendocrine cell markers may exist.     

Urothelial carcinoma with oncocytic features: an extremely rare case presenting a diagnostic challenge in urine cytology

Recognizing histological variants in urothelial carcinoma (UC) is important because some may be associated with different clinical outcomes and/or therapeutic approaches; being aware of unusual histological variants may also be crucial in preventing diagnostic misinterpretations. Histological variants based on cytoplasmic features, such as clear-cell, plasmacytoid, rhabdoid, and lipoid-rich variants, are described in invasive UC; however, these cytoplasmic features are not formally defined and not usually encountered in non-invasive UC. Oncocytic cytoplasm has not been well described in either invasive or non-invasive UC. Herein, we report an exceedingly rare case of UC with oncocytic features arising in the right renal pelvis, which presented a diagnostic challenge in urine cytology due to the relatively low nuclear-to-cytoplasmic ratio; however, it could definitively be diagnosed using histological specimens. UC diagnosis is based on the presence of papillary architecture and widespread p53 nuclear accumulation, suggesting malignancy. An oncocytic tumor is generally considered to be not actively dividing, as shown by the low Ki-67 labeling index in this case. In spite of the low proliferative activity, the possibility of intravesicle recurrence (IVR) should be considered since positive preoperative cytology of upper tract UC is a risk factor for IVR after nephroureterectomy.     

Malignant intraductal oncocytic papillary neoplasm of the common bile duct

Recently, several cases of intraductal oncocytic papillary neoplasm (IOPN) of the liver and hepatic bile ducts have been reported. The author herein reports the first case of IOPN of the common bile duct (CBD). A 78-year-old man was admitted to our hospital because of jaundice. Imaging modalities including US, CT, MRI revealed an intraductal tumor of the middle CBD and biliary dilation distal to the tumor. A partial resection of the CBD was performed. Grossly, a papillary tumor measuring 20 × 15 mm was found within the CBD. Mucus is absent. Histologically, the papillary tumor was composed of atypical oncocytes. The atypia was enough to be diagnosed as adenocarcinoma. No invasive features were noted. Immunohistochemically, the tumor cells were positive for pancytokeratins (CK), CK 7, CK 18, CK19, EMA, CA19-9, CEA, mitochondria, p53 protein, C-erbB2, Ki-67 (labeling = 80%), MUC2, MUC5AC and MUC-6,. The tumor cells were negative for CK8, CK20, chromogranin, synaptophysin, neuron-specific enolase, S100 protein, CD56, MUC1, CD10 and CDX2. These immunohistochemical findings were compatible with IOPN. The patient died of other non-tumorous disease 7 year after the operation. In summary, the author presented the first case of IOPN of the CBD.     

Inflammatory myofibroblastic tumor with ALK/TPM3 fusion presenting as ileocolic intussusception: an unusual presentation of an unusual neoplasm

Inflammatory myofibroblastic tumor is a rare spindle cell lesion of indeterminate malignant potential occurring in both pulmonary and extrapulmonary tissues. This report describes an unusual presentation of an unusual tumor at an unusual location: an intramural ileal case of inflammatory myofibroblastic tumor presenting with intussusception in a 29-year-old woman. We characterize this tumor through microscopic and ultrastructural analysis, extensive immunohistochemical analysis, ploidy analysis, and Epstein-Barr virus in situ hybridization, and we report the finding of an ALK/TPM3 fusion using fluorescence in situ hybridization.     

Cytologic imprints of giant atypical bronchopulmonary carcinoid tumor of the lung with extensive oncocytic component

Bronchopulmonary carcinoid tumors are found in less than 2 of 100,000 people yearly and comprise approximately 1-2% of all lung neoplasms. They are usually diagnosed in the fifth decade of life and occur more frequently in women. Most are central and endobronchial in location. The symptoms presented are those associated with bronchial obstruction, such as coughing, wheezing, and dyspnea. Over one-third of patients are asymptomatic and have an incidentally detected peripheral pulmonary nodule. Diagnosis is usually made by bronchoscopy with bronchial brushings or biopsy. We present the cytologic imprint findings of a case of a 66-year-old man with an atypical giant bronchopulmonary carcinoid with extensive oncocytic component, who underwent a total right pneumonectomy.     

Catecholamine secreting tumor of the glomus jugulare

Summary A case of endocrinologically active glomus jugulare is presented. The localization within the tumor of the catecholamine hyperproduction was demonstrated by selective venous sampling from the vena jugularis externa draining the tumor.The norepinephrine/epinephrine ratio was high and of the same order in blood, urine and cerebrospinal fluid. Bioassay showed high levels of intratumoral norepinephrine and epinephrine.The ways of treatment and their influence on the catecholamines are briefly discussed. In every phaeochromocytoma-like syndrome both the existence of catecholamine hyperproduction and the anatomical site of this hypersecretion have to be precisely documented in order to define the correct treatment to be applied.     

Malignant glomus tumor in the branchial muscle of a 16-year-old girl

Malignant glomus tumor is an extremely rare neoplasm and its histological features are not well characterized. We report a 16-year-old female patient with a malignant glomus tumor. The patient was admitted to our hospital presenting with a mass in the right upper arm that she had noticed for the previous 6 months. Computed tomography and magnetic resonance imaging revealed an expanded mass involving the surrounding tissues. At surgery, an ill-defined and expanded mass was found, 5 x 4 x 3 cm in size, in the right branchial muscle. The tumor was extirpated, along with neighboring muscle tissues. Histologically, tumor cells were round to short-spindle shaped, forming solid sheets admixed with vessels of varying size. Their nuclei were uniformly oval to round, and their cytoplasms were slightly eosinophilic. The growth pattern of the tumor cells resembled that of glomus tumor, but mitotic figures were frequent (as high as 10 per 10 high-power fields). Immunohistochemically, the tumor cells were positive for vimentin and muscle actin, but negative for desmin. There were no areas typical of benign glomus tumor or sarcomatous change. These findings led us to a diagnosis of primary malignant glomus tumor arising de novo. There has been no recurrence or metastasis for 21 months after wide excision.     

胃肠道血管球瘤15例临床病理学分析

目的探讨胃肠道血管球瘤(GIGT)的组织病理学特征、诊断及鉴别诊断。方法收集郑州大学第一附属医院2011年1月至2018年6月诊断的15例GIGT,分析其临床病理特征、免疫表型、BRAF基因突变及预后,并复习相关文献。结果15例GIGT,男性7例,女性8例。14例发生于胃,1例发生于回肠。患者年龄37~59岁,中位年龄49岁,平均50岁。11例胃血管球瘤以间断腹痛、腹胀入院,另3例于体检时发现胃窦部占位入院;1例回肠血管球瘤以腹痛伴大便带血入院。影像学检查,胃血管球瘤位于黏膜下,边界清楚,动脉期强化明显;肠血管球瘤累及局部肠壁全层并堵塞管腔。大体检查:瘤体最大径1.5~3.0 cm不等,平均2.3 cm,切面实性。显微镜下观察:胃血管球瘤位于固有肌层间,富血管,与周围分界清楚,但均无明显包膜形成;瘤细胞大小一致,呈圆形或卵圆形,围绕血管周围呈血管外皮瘤样或实性巢团状排列;细胞形态温和,核圆形居中,染色质细腻,核仁不明显,核分裂象难见,无坏死,其中2例局部见小灶状钙化;2例见肿瘤侵入黏膜层;2例见脉管瘤栓;5例见神经侵犯。肠血管球瘤累及肠壁全层,细胞密度高,异型性明显,核分裂象热点区约(5~6)个/HPF。免疫组织化学显示,瘤细胞弥漫强表达平滑肌肌动蛋白(SMA)和Ⅳ型胶原,部分区域中等强度表达Caldesmon或Calponin,有12例局部弱表达突触素。胃血管球瘤的Ki-67阳性指数1%~30%不等,均值约6%。肠血管球瘤的Ki-67阳性指数约70%。15例GIGT均未检测到BRAF基因V600E位点突变。所有患者手术治疗后均未行放化疗。电话随访到13例患者,随访时间18~90个月,平均42个月,12例胃血管球瘤患者均健存,肠血管球瘤患者术后15个月出现肝转移。结论血管球瘤是胃肠道少见的间叶源性肿瘤,应与胃肠道间质瘤、神经内分泌肿瘤、平滑肌瘤、孤立性纤维性肿瘤及副节瘤等鉴别。多数GIGT生物学行为良善,预后良好,但尚需长期临床随访。     

Adrenocorticotropic hormone‐producing thymic neuroendocrine carcinoma with oncocytic features: A case report and review of literature

Thymic neuroendocrine carcinomas are the most common mediastinal neuroendocrine tumor. These malignancies are not often diagnosed by fine‐needle aspiration (FNA), as they are more commonly diagnosed by biopsy or excision. We describe a case of a FNA of a paratracheal mass from a 38‐year‐old man who presented with Cushing syndrome. A low‐grade neuroendocrine carcinoma with oncocytic features was diagnosed, which was later confirmed by excision of the thymus, anterior mediastinal and paratracheal soft tissue, and lymph nodes. Oncocytic features in these tumors are a rare finding and bring metastatic medullary thyroid carcinomas as well as other metastases into the differential diagnosis. The prognosis of neuroendocrine carcinomas in this location is worse than neuroendocrine carcinomas in other areas, and close follow‐up is recommended. Diagn. Cytopathol. 2015;43:329–334. © 2014 Wiley Periodicals, Inc.