Retroperitoneal epithelioid angiomyolipoma leading to fatal outcome

Abstract

Epithelioid angiomyolipoma (AML) is a newly established variant of AML, characterized by monomorphous epithelioid cells that show HMB-45 immunopositivity, and it often displays aggressive behavior. To date, they have mostly appeared in the kidneys; however, the present autopsy case of a 43-year-old female without the stigmata of tuberous sclerosis complex had a huge retroperitoneal mass, accompanied by involvement of the regional lymph nodes. Histopathologically, the tumor was composed of round, polygonal or short spindle-shaped monomorphous cells with abundant eosinophilic cytoplasm and large nuclei with frequent multinucleation. Mitotic figures were scattered. Mature fat cells and thick-walled abnormal blood vessels were totally absent. Immunohistochemically, the tumor cells were reactive with HMB-45 and alpha-smooth muscle actin antibodies. In spite of curative surgery and repeated radio- and chemotherapy, the tumor continued to grow and brought about the patient's death 4 years after the initial symptoms. At autopsy, the peritoneal cavity was filled with the tumor mass exceeding 5.5 kg. Histopathological features were essentially the same as those of biopsy samples, but the cellular pleomorphism and emperipolesis were more easily identified. This report calls attention to this unusual manifestation of AML in the retroperitoneum and the importance of distinguishing it from sarcomas and/or paragangliomas.

Reasons to reconsider the significance of apoptosis for cancer therapy

“…into Chinese hamster cells (for a review, see Ikeuchi et al 1972). Likewise, it is well documented that multinucleated cells appear after ionizing radiation in vitro as well as in vivo (Ikeuchi et al 1972, Bhattathiri et al 1998a, b, Somosy 2000 or in tumours of different origin (Yokoo et al 2000, Hosaka et al 2001, Jagielski et al 2001. Several mechanisms are under discussion, e.g.…”

Fatal Malignant Metastastic Epithelioid Angiomyolipoma Presenting in a Young Woman: Case Report and Review of the Literature

“…But there have been reported poor responses to dacarbazine, carboplatin, cyclophosphamide as well as dacarbazine, ifostamide and mesna and no response reported for a patient receiving radiation. 9,10 There has been scant published data in regard to chemoradiation treatment likely due to the rarity of the disease as well as due to the emergence of other modes of treatment.…”

Malignant Monotypic Epithelioid Angiomyolipoma of the Retroperitoneum

“…Aside from the present case, there appears to be only 1 additional report in which an extrarenal retroperitoneal AML was shown to exhibit aggressive behavior. Yokoo et al [2] described a 43-yearold woman with a large retroperitoneal tumor composed of HMB-45-positive epithelioid cells who developed multiple pelvic recurrences over 4 years despite radiation and chemotherapy. Postmortem examination revealed no evidence of distant metastatic disease, though an AML of conventional histology was discovered in the right kidney, as well as a hepatic nodule of AML with predominantly epithelioid features.…”

“…The pleomorphic epithelioid cells may simulate the appearance of a number of different neoplasms including poorly differentiated carcinoma, sarcoma, and melanoma. Immunohistochemistry is a useful means of differentiating these entities, as the epithelioid cells of AML are characteristically positive for HMB-45 and negative for epithelial markers [2,4,5,[7][8][9][10]12,[26][27][28][29][30][31], which serves to distinguish them from most other neoplasms. Immunoreactivity for HMB-45 may correspond to the presence of premelanosome-like structures that have be identified ultrastructurally within the epithelioid cells [30][31][32].…”