Topic
Glomangiosarcoma
About: Glomangiosarcoma is a research topic. Over the lifetime, 57 publications have been published within this topic receiving 1923 citations. The topic is also known as: malignant Glomus neoplasm & Glomangiosarcoma.
Papers published on a yearly basis
Papers
TL;DR: Using this classification scheme, metastasis was observed in 38% of tumors fulfilling the criteria for malignancy, and metastatic disease was not seen in any specimen classified as symplastic glomus tumor, glomu tumor of uncertain malignant potential, or glomangiomatosis.
Abstract: Occasional glomus tumors display unusual features, such as large size, deep location, infiltrative growth, mitotic activity, nuclear pleomorphism, and necrosis. Although a small number of purportedly malignant glomus tumors have been described, histologic criteria for malignancy in glomus tumors have never been elaborated. The authors studied 52 unusual glomus tumors (retrieved from their consultation files) previously diagnosed as "atypical" or "malignant" by virtue of nuclear atypia, infiltrative growth, or mitotic activity. They evaluated size, depth, growth pattern, cellularity, nuclear grade, number of mitotic figures per 50 high-power fields (HPF), atypical mitotic figures, vascular space involvement, and necrosis to define criteria for malignancy in glomus tumors. Estimated relative risk was calculated and the Fisher exact test was used for statistical analysis. The 27 female patients and the 25 male patients ranged in age from 8 to 83 years (median age, 43 years). The tumors measured from 0.2 to 12 cm (median size, 2 cm) and occurred predominantly in the extremities, in both the superficial (n = 35) and deep (n = 17) soft tissues. Atypical features were usually observed centrally with a rim of benign-appearing glomus tumor. Follow-up information (n = 35; range, 5 months-23 years; mean 5.5 years) showed seven recurrences, eight metastases, and seven deaths from disease. Five-year cumulative metastatic risk increased significantly for tumors with a deep location (p = 0.005), with a size of more than 2 cm (p = 0.004), and with atypical mitotic figures (p = 0.004). Mitotic activity of more than 5 mitoses/50 HPF, high cellularity, the presence of necrosis, and moderate to high nuclear grade approached but did not reach significance. High nuclear grade alone, infiltrative growth, and vascular space involvement were not associated with metastasis. The authors propose the following classification scheme and criteria. Malignant glomus tumor: Tumors with a deep location and a size of more than 2 cm, or atypical mitotic figures, or moderate to high nuclear grade and > or =5 mitotic figures/50 HPF. Symplastic glomus tumor: Tumors with high nuclear grade in the absence of any other malignant feature. Glomus tumor of uncertain malignant potential: Tumors that lack criteria for malignant glomus tumor or symplastic glomus tumor but have high mitotic activity and superficial location only, or large size only, or deep location only. Glomangiomatosis: Tumors with histologic features of diffuse angiomatosis and excess glomus cells. Using this classification scheme, metastasis was observed in 38% of tumors fulfilling the criteria for malignancy. In contrast, metastatic disease was not seen in any specimen classified as symplastic glomus tumor, glomus tumor of uncertain malignant potential, or glomangiomatosis.
647 citations
TL;DR: Six cases of locally aggressive and/or potentially malignant glomus tumors are described and the most difficult to recognize is the de novo glomangiosarcoma (GADN), which must be distinguished from other round cell sarcomas.
Abstract: Six cases of locally aggressive and/or potentially malignant glomus tumors are described. On the basis of clinical and pathologic criteria, the following classification is proposed. The first category is a locally infiltrative glomus tumor (LIGT) which has the usual glomus histologic features. The second group is a cytologically malignant tumor arising and merging with a typical glomus tumor, designated glomangiosarcoma arising in a benign glomus (GABG). The third category and the most difficult to recognize is the de novo glomangiosarcoma (GADN), which must be distinguished from other round cell sarcomas. Most of these locally aggressive glomus tumors are vimentin positive and are immunoreactive for muscle-specific actin. Electron microscopic examination in one GABG case showed cells with numerous microfilaments and pinocytotic vesicles; a second GADN case contained cells with microfilaments and an incomplete basal lamina. As a group these locally aggressive or potentially malignant glomus tumors are larger and more deeply located than the conventional glomus tumor. Although 50% of these tumors recurred locally, none have metastasized.
172 citations
TL;DR: These tumors show a variety of clinical and pathologic differences from the more common cutaneous variety, including later age at presentation, larger size, and more frequent atypical/malignant features.
Abstract: Pulmonary and mediastinal glomus tumors are rare lesions, with four previously reported primary pulmonary cases and three mediastinal cases. The authors report one mediastinal glomus tumor, a locally infiltrative type, and four pulmonary glomus tumors, including the first case of primary pulmonary glomangiosarcoma. These tumors show a variety of clinical and pathologic differences from the more common cutaneous variety, including later age at presentation, larger size, and more frequent atypical/malignant features. Mediastinal and pulmonary glomus tumors both have an average patient age at presentation of 45 years. However, compared with their pulmonary counterparts, mediastinal glomus tumors are less common, more often symptomatic, and are larger (average size, 5.4 cm). Additionally, mediastinal glomus tumors more often demonstrate malignant or atypical features. Pulmonary glomus tumors average 3.3 cm in greatest dimension, with the majority measuring less than 2.5 cm. The pulmonary glomangiosarcoma presented was large, measuring 9.5 cm, and showed increased mitotic count (9 mitoses/10 high-power fields), necrosis, cytologic atypia, and was associated with disseminated disease. Regardless of clinical symptoms, histologic features, and even metastases, the vast majority of all benign and malignant glomus tumors are indolent and cured surgically, with adjuvant therapy needed only for occasional patients with more advanced disease. The four patients with glomus tumors reported are currently alive and free of disease as of last follow up. The patient with the glomangiosarcoma developed widespread metastases and died of disease 68 weeks after initial therapy.
149 citations
TL;DR: Trends in the clinical versus pathologic diagnoses of glomus tumor, common competing diagnoses, a difference in demographics than is commonly reported, and important differences in the use adjunctive diagnostic tools including radiology and immunohistochemistry are illustrated.
Abstract: Background. Glomus tumors are relatively uncommon subcentimeteric benign perivascular neoplasms usually located on the fingers. With their blue-red color and common subungual location, they are com...
142 citations
TL;DR: A case of widespread metastases of a malignant glomus tumor involving the skin, lungs, jejunum, liver, spleen, and lymph nodes in a 63-year-old man with end-stage chronic renal failure is reported.
Abstract: Glomus tumors are characteristically benign solitary tumors. A few cases of malignant glomus tumors have been reported; however, they are usually only locally invasive, and metastases are exceedingly rare. We report a case of widespread metastases of a malignant glomus tumor involving the skin, lungs, jejunum, liver, spleen, and lymph nodes in a 63-year-old man with end-stage chronic renal failure. At autopsy, multiple glomus body hamartomas were also noted throughout the dermis proximal to the skin tumors. Histologically, the tumor was composed of monotonous round cells in solid sheets and investing numerous capillary-sized vessels. Approximately four to six mitoses were present per high-power field. The tumor cells were strongly immunoreactive for actin and vimentin, and ultrastructurally there were whorls of microfilaments with focal condensations. This case is exceptional because of its aggressive course.
99 citations
Related Topics (5)
Performance Metrics
| Year | Papers |
|---|---|
| 2021 | 3 |
| 2020 | 2 |
| 2019 | 1 |
| 2018 | 3 |
| 2017 | 1 |
| 2016 | 4 |