Kolm, Isabel;
Konstantinova, Anastasia M.;
Kutzner, Heinz;
Barghorn, Andre;
Vanecek, Tomas;
Mangana, Joanna;
Kazakov, Dmitry V.
Cuticular Poroma: A Rare Poroma Variant Simulating a Malignant Neoplasm That Often Harbors YAP1::NUTM1 Fusions Similar to Their Conventional Counterparts
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Medientyp:
E-Artikel
Titel:
Cuticular Poroma: A Rare Poroma Variant Simulating a Malignant Neoplasm That Often Harbors YAP1::NUTM1 Fusions Similar to Their Conventional Counterparts
Beteiligte:
Kolm, Isabel;
Konstantinova, Anastasia M.;
Kutzner, Heinz;
Barghorn, Andre;
Vanecek, Tomas;
Mangana, Joanna;
Kazakov, Dmitry V.
Beschreibung:
<jats:title>Abstract:</jats:title>
<jats:p>Cuticular poroma is a rare variant of poroma composed of exclusively or predominantly cuticular cells, namely of large cells with ample eosinophilic cytoplasm. We report 7 cases of this rare tumor identified among 426 neoplasms diagnosed as poroma or porocarcinoma. The patients were 4 males and 3 females, ranging in age from 18 to 88 years. All presented with a solitary asymptomatic nodule. The location included knee (2 cases), shoulder, thigh, shin, lower arm, and neck (each 1). All lesions were surgically removed. No evidence of disease was observed in 5 patients with available follow-up (range 12–124 months).</jats:p>
<jats:p>Microscopically, all neoplasms were composed of variably sized, focally closed packed, or interconnecting nodules constituted mostly of cuticular cells. Small poroid cells were a focal feature in 5 tumors, whereas in the remaining 2 cases, poroid cells with conspicuous but still in minority. Five neoplasms were somewhat asymmetric, with irregular outlines. Ductal differentiation and intracytoplasmic vacuoles were seen in 6 tumors. Other features variably encountered were conspicuous intranuclear pseudoinclusions, cystic change, occasional multinucleated cells, increased mitoses, and stromal desmoplasia. Four of the 5 tumors analyzed with next-generation sequencing yielded <jats:italic toggle="yes">YAP1::NUTM1</jats:italic> fusions. In addition, various mutations, mostly of unknown significance were identified in one neoplasm.</jats:p>