Angiomyolipoma predominantly composed of smooth muscle cells: problems in histological diagnosis

Nonomura, Akitaka; Minato, Hiroshi; Kuroda, Hiroshi

doi:10.1046/j.1365-2559.1998.00426.x

Cited by 77 publications

(67 citation statements)

References 19 publications

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“…Recent reports have considerably widened the spectrum of PEC lesions reporting them in the uterus, pancreas and in the falciform ligament (7)(8)(9)(10)(11). PEC tumors can mimic renal clear cell carcinoma (12)(13)(14)(15)(16)(17) epithelioid leiomyosarcoma (18), hepatocellular carcinoma (19,20) conventional leiomyosarcoma or other high grade sarcomas.…”

Section: Discussionmentioning

confidence: 99%

Abdominopelvic Sarcoma of Perivascular Epithelioid Cells. Report of Four Cases in Young Women, One with Tuberous Sclerosis

et al. 2001

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The perivascular epithelioid cell has been proposed to be the unifying proliferating cell type in a number of lesions such as angiomyolipoma, lymphangiomyomatosis, clear cell "sugar" tumor and renal capsuloma. With the exception of rare examples of angiomyolipoma, they are nonmetastasizing. We report four examples of a new member of this family of perivascular epithelioid cell neoplasms that occur in abdominopelvic location and show metastatic properties. The patients, all women, were aged 19 to 41 years (mean, 32), and presented with a tumor mass involving the serosa of the ileum, uterus or pelvic cavity. Morphologically, the tumors were composed of sheets of large polygonal cells with glycogen-rich clear or eosinophilic cytoplasm and moderately pleomorphic nuclei, traversed by a delicate vasculature, mimicking clear cell carcinoma. There were areas of coagulative necrosis and occasional mitotic figures. Intracytoplasmic brown pigment was present in two cases. Spindly cells, smooth muscle and fat were absent. Lymphovascular invasion was present in all, lymph node metastasis was documented in two and metastasis to the ovary was present in one case. Two patients developed widespread metastatic disease after 10 and 28 months from diagnosis. One patient showed the clinical signs of tuberous sclerosis. In spite of the epithelial-like appearance, the tumor cells were negative for epithelial markers but were strongly positive with the melanogenesis-related marker HMB45. Another melanogenesis marker (MART-1) was positive in two cases. Other markers including S-100 protein, vimentin, muscle-specific actin, desmin and chromogranin A were negative. Thus, these tumors are not readily classifiable in the existing schema of known entities, and show overlapping morpho-phenotypic features of clear cell "sugar" tumor of the lung and epithelioid angiomyolipoma. We consider them as sarcomas composed of a pure population of uncommitted perivascular epithelioid cell, that lack modulation toward smooth muscle or adipose cells.

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Section: Discussionmentioning

confidence: 99%

Abdominopelvic Sarcoma of Perivascular Epithelioid Cells. Report of Four Cases in Young Women, One with Tuberous Sclerosis

et al. 2001

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“…Although specific histologic features have been described, immunohistochemistry also is helpful for its diagnosis, including negative reactivity with anticytokeratin antibodies, positive reaction with antibodies to actin, and consistent expression of melanogenic markers, particularly HMB-45, tyrosinase, and microphthalmia transcription factor (50). Other unusual forms of angiomyolipoma include the lipomatous and leiomyomatous variants, which resemble lipoma or well-differentiated liposarcoma, and leiomyoma or leiomyosarcoma, respectively (51)(52)(53).…”

Section: Discussionmentioning

confidence: 99%

Concurrent Angiomyolipoma and Renal Cell Neoplasia: A Study of 36 Cases

et al. 2001

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Little is known about the association of angiomyolipoma and adult renal-cell neoplasia. We studied the clinicopathologic features of 36 patients with concurrent angiomyolipoma and renal-cell neoplasia from the consultation and surgical pathology files of nine institutions. HMB-45 immunoreactivity was analyzed in both neoplasms. Twenty-five sporadic cases of patients with angiomyolipoma and renal-cell neoplasia and 11 cases of patients with tuberous sclerosis, as defined by Gomez' criteria, had mean ages of 59 and 53 years, respectively, and female-male ratios of 2:1 and 5:1, respectively. The mean size of the angiomyolipomas was 1 cm in the sporadic cases and 3 cm in those patients with tuberous sclerosis (medians: 0.5 and 3 cm, respectively, P ‫؍‬ .002). The mean sizes of the renal-cell neoplasms were 5 cm in sporadic cases and 6 cm in patients with tuberous sclerosis (medians: 4 and 5 cm, respectively; P ‫؍‬ .88). In both clinical settings, angiomyolipoma was more commonly the incidental tumor. Clear-cell (conventional) renal-cell carcinoma was the most common renal-cell neoplasm in both groups of patients, accounting for approximately two thirds of the tumors. In patients with tuberous sclerosis, 27% of renal-cell neoplasms were oncocytomas, compared with 8% in sporadic cases (P ‫؍‬ .15). Papillary neoplasia, chromophobe, and collecting-duct renal-cell carcinoma were found only in sporadic cases. All of the 22 renal-cell neoplasms studied were negative for HMB-45, whereas all 25 angiomyolipomas studied were positive.

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“…In cases of ambiguity, reactivity for HMB-45 confirms the diagnosis [2]. This however does not reconcile with a mesenchymal origin of the epitheloid or spindle cells.…”

Section: Discussionmentioning

confidence: 87%