Cutaneous angiosarcoma of the scalp mimicking a keratoacanthoma.

Cutaneous angiosarcoma (CA) has a wide range of clinical presentations. In this case report, we discuss a 78-year-old gentleman, who presented with a keratoacanthoma-like scalp lesion that turned out histologically to be a cutaneous angiosarcoma. A brief overview of CA, including its etiology, prognostic factors, clinical manifestations, and treatment options will also be discussed.

T-cell-rich angiomatoid polypoid pseudolymphoma of the skin: a clinicopathologic study of 17 cases and a proposed nomenclature.

BACKGROUND: We describe a series of previously unreported, distinctive, polypoid solitary T-cell-rich cutaneous pseudolymphomas. METHODS: The clinicopathologic features were examined in 17 cases. RESULTS: Patient ages ranged from 16 to 71 years (mean = 38.5) with a female predominance (female : male = 14 : 3). All lesions, clinically diagnosed most often as pyogenic granuloma, presented as a solitary, polypoid, erythematous, papule ranging in size from 2.5 to 7.5 mm (mean = 5.8). Most occurred on the head and neck (7) and trunk (6) with other sites including the thigh (1), shoulder (1) and knee (1). A dense dermal infiltrate composed of mildly atypical lymphocytes with variable numbers of admixed plasma cells and histiocytes was prototypical. Commonly, there was an associated epidermal collarette (16/17), Grenz zone (11/17) or admixed eosinophils (8/17). Prominent vessels lined by plump endothelial cells, reminiscent of high endothelial venules of lymph nodes, were universal and some degree of telangiectasia was also common (12/17). CD3-positive T-cells consisted of an admixture of CD4-positive and CD8-positive forms (15/16). Multiple studies suggested polyclonality (seven cases). No recurrences after lesional excision were noted in the 17 patients with a follow-up range from 24 to 120 months (mean = 46.6). CONCLUSION: Although these lesions share histopathologic features of the so-called acral pseudolymphomatous angiokeratoma of children (APACHE), they occur in a completely different clinical setting, present in solitary and polypoid fashion and are T-cell rich. We propose the diagnostic label T-cell-rich angiomatoid polypoid pseudolymphoma for this distinctive but presumably reactive lesion.

Mucinous hidradenoma: a report of three cases.

Mucinous cutaneous adnexal tumors are an uncommon occurrence. We describe three cases of mucinous hidradenoma characterized by diffuse and prominent mucinous cell proliferation and discuss the relevant literature. The patients (two men and one woman) ranged in age from 32 to 72 years. The lesions were completely excised, and all patients were alive without evidence of disease (follow-up interval 3-4 years). Case 1 presented as a 1.3-cm eyelid nodule and histology showed a mucinous apocrine hidradenoma with a proliferation of mucinous cells and apocrine glands. Case 2, from the shoulder of a 40-year-old man, was a 2.5-cm well-circumscribed nodule composed of hyperplastic villoglandular proliferation of mucinous cells with focal squamous whorls and focal columnar glandular cells showing decapitation secretion. The tumor was also interpretated as a mucinous hidradenoma, most likely of follicular-apocrine origin. Case 3 was a 3.0-cm scalp nodule. The histology showed a mucinous clear-cell hidradenoma composed of numerous mucinous glands amidst small squamous cells with clear cytoplasm and a hyalinized fibrovascular stroma.

Sarcomatoid eccrine porocarcinoma: report of two cases and a review of the literature.

Eccrine porocarcinoma is an uncommon sweat gland malignancy. To the best of our knowledge, there has been no report in the English literature of porocarcinoma with predominantly undifferentiated sarcomatous change. We present two cases of sarcomatoid eccrine porocarcinoma associated with a benign poroma. Case 1 pertained to an 82-year-old woman with an ulcerated chest wall tumor, and Case 2 was that of a 74-year-old woman who presented with an ulcerated plaque in the lower leg. Case 1 showed an unusual pseudo-angiosarcomatous morphology with spindle cells dissecting through collagen bundles and forming vascular like channels. Case 2 revealed high-grade malignant spindle cells with focal evidence of ductal differentiation. In both the cases, benign poromatous elements were histologically evident. Immunohistochemistry performed showed pancytokeratin positivity in spindle cells of both lesions. Epithelial membrane antigen and carcino-embryonic antigen positivity in the malignant ductal elements and focal smooth muscle actin staining of the spindle cells were demonstrated in Case 2. A brief review of relevant literature is presented.

Symplastic hemangioma: report of two cases.

Symplastic hemangioma is characterized by degenerative atypia of vascular smooth muscle and interstitial cells within a pre-existing vascular lesion with minimal endothelial cell atypia. We describe an additional two cases of this distinctive but poorly recognized entity. On histology, both lesions revealed a cirsoid aneurysm-type appearance with thick-walled and variably dilated blood vessels. The vascular endothelial cells showed mild nuclear hyperchromasia with no multilayering or mitoses. The atypical cells, either located within the vascular smooth muscle wall or within the interstitium, were spindle or epithelioid with varying degrees of hyperchromasia, nuclear enlargement, pleomorphism, and multinucleation. Perivascular hemorrhage, vascular thrombosis, and focal papillary endothelial hyperplasia were uniformly present. The variably fibrous to edematous stroma showed hemosiderin deposits and a mononuclear inflammatory infiltrate. Clusters of adipocytes were present within the superficial dermis. Rare atypical mitoses and occasional bizarre lipoblast-like stromal cells were identified in one tumor. Immunohistochemistry showed focal smooth muscle actin positivity in the pleomorphic cells of the vascular walls. CD68 and CD34 stained occasional stromal cells in the interstitial location. Both the cases showed no recurrence. The bizarre cytologic changes are interpreted as degenerative in nature and probably akin to that observed in ancient schwannoma and uterine symplastic leiomyoma.

Uterine epithelioid endometrial stromal sarcoma presenting as a "cervical polyp".

Although appearance of conventional uterine endometrial stromal sarcoma is easily recognized on histology, it may uncommonly assume unusual appearances such as uterine tumor resembling ovarian sex-cord tumor, thereby hindering its diagnosis. Recently, its manifestation as an epithelioid neoplasm was described. In this report, we detail yet another instance where this tumor adopted an epithelioid morphology, presenting itself as a polyp extruding from the cervical os in a 41-year-old Chinese woman. Both the polypectomy and subsequent hysterectomy specimens revealed a predominant proliferation of CD10-negative, caldesmon-negative, and CD117-positive epithelioid cells set within a stroma containing vascular proliferation resembling endometrial stromal tumor. Areas of typical low-grade endometrial stromal sarcoma containing spindle cells that were focally positive for CD10 and negative for CD117 were present in close association with the epithelioid areas. The differential diagnoses and possible implication of CD117 positivity are discussed.

Role of intraoperative smear cytology in the diagnosis of anaplastic oligodendroglioma. A case report.

BACKGROUND: While the intraoperative smear cytology of oligodendroglioma is well documented, the cytologic features of anaplastic oligodendroglioma have been described rarely. CASE: A 41-year-old man with a previous history of a brain tumor five years earlier presented with recurrent neurologic symptoms. Radiologic examination revealed a predominantly cystic tumor with solid areas and calcifications arising from the left temporal lobe. Intraoperative smears of the tumor were hypercellular, disclosing loosely cohesive and single cells resembling oligodendrocytes with mitotic activity and moderate anisonucleosis. The background displayed a characteristic vacuolated appearance. Tissue submitted for frozen section showed a tumor with an infiltrative margin with areas of perineuronal satellitosis. CONCLUSION: A diagnosis of anaplastic oligodendroglioma can be made intraoperatively with the aid of smear cytology in conjunction with frozen section, assisting intraoperative management.

Two cases of epithelioid angiosarcoma involving the thyroid and a brief review of non-Alpine epithelioid angiosarcoma of the thyroid.

Epithelioid angiosarcoma involving the thyroid is a rare entity, more often described in the Alpine region. Two cases of epithelioid angiosarcoma that affected the thyroid in patients from a non-Alpine location were diagnosed during a 10-year period in our department. The first case occurred in an 89-year-old Chinese man with a history of longstanding goiter, whereas the second case involved a 74-year-old Chinese man with a history of angiosarcoma of the scalp. On histologic examination, both thyroid tumors were composed of plump epithelioid cells with vesicular chromatin and prominent nucleoli, forming vascular structures and solid sheets. Positive staining for CD31 and factor VIII-related antigen confirmed endothelial differentiation in both cases. Both patients died within 5 months following the diagnosis of thyroid disease. The relationship of the scalp angiosarcoma and thyroid disease of the second patient is unclear. A brief review of non-Alpine primary thyroid epithelioid angiosarcoma is presented.

Role of FNAC in metastasizing malignant mixed tumor of the external auditory canal. A case report.

BACKGROUND: No previous report of metastasizing mixed tumor (pleomorphic adenoma) of the external auditory canal (EAC) has been described. CASE: A 12-year-old, Chinese girl with a history of mixed tumor of the EAC presented with a locally recurrent, aggressive tumor and metastases to the lung and bone five years later. The primary, locally recurrent and metastatic lung tumor showed epithelial and myoepithelial elements with duct formation, chondromyxoid stroma and mitotic activity in the cellular areas on histology. Fine needle aspiration cytology (FNAC) disclosed the presence of spindle cells blending into chondromyxoid fibrillar ground substance in the recurrent and metastatic lung tumors. CONCLUSION: In primary mixed tumor of the EAC, FNAC plays a useful role in the diagnosis of recurrent and metastatic disease. Its ability to identify ominous features, such as increased mitoses in this case, may be limited by sampling. Since cytology and histology cannot reliably prognosticate, long-term follow up of mixed tumor of the EAC after complete excision is advocated.

Solitary fibrous tumour in the deep soft tissue of the neck in a Chinese man.

Originally described in the pleura, solitary fibrous tumour (SFT) is now reported in a variety of extrapleural sites. However, description of SFT in the deep soft tissue of the neck is very rare. In this report, we document the neoplasm, deep to the right platsyma muscle but superficial to the deep fascia just above the clavicle, in an otherwise well 50-year-old Chinese man. Histological examination of the excised specimen disclosed spindle-shaped cells disposed in short fascicles as well as randomly in association with areas of varying cellularity, keloidal hyalinisation of the stroma and haemangiopericytoma-like vessels. The tumour cells were CD34-, bcl-2- and CD99-positive. Since histological features may not accurately predict the biological behaviour of this tumour, careful long-term follow-up is advocated.

Intravascular lymphomatosis of the lung and liver following eyelid lymphoma in a Chinese man and review of primary pulmonary intravascular lymphomatosis.

Intravascular lymphomatosis (IVL) is characterised by an almost exclusive intravascular proliferation of malignant lymphoid cells, with the diagnosis often made only when the illness is in its terminal phase or at autopsy. We detail a case of IVL affecting the lung and liver of a 49-year-old Chinese man presenting primarily with lung symptoms and incidental findings of abnormal serum transaminase levels, the ante-mortem diagnosis being established on transbronchial lung biopsy and percutaneous liver biopsy specimens, respectively. Histology disclosed CD20 + CD5 - CD10 [corrected] - malignant large mononuclear B cells within the lumina of the blood vessels of the affected organs as well as sinusoids of the liver. Significantly, the patient had a history of large B cell lymphoma affecting the eyelid 18 months prior to the angiotropic disease, suggesting a possible link between the more common types of non-Hodgkin's lymphoma and IVL. A brief review of all cases of primary pulmonary intravascular lymphomatosis is also presented.