PMC6955122_01
(a) The right ovary measures 3.4 x 2.4 x 2.2 cm and shows diffuse abnormal hypoechogenicity and increased vascularity. (b) The left ovary measures 2.8 x 2.2 x 2.1 cm and shows diffuse abnormal hypoechogenicity with mildly increased vascularity. (c) The cervix and uterus are enlarged and heterogeneous with multifocal hypodense and cystic foci. An irregular hypodense lesion in the anterior uterine body measuring 5.1 x 8.0 x 4.0 cm is present, which invades the right lateral uterine wall. There is associated adjacent pelvic ascites. (d) There is a hyperdense, enhancing lesion in the jejunum in the left hemiabdomen with central hypoattenuation measuring 2.2 x 2.0 x 2.2 cm.
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An ultrasound of the pelvis showed enlarged ovaries that were hypoechoic with increased vascularity concerning for a neoplasm (Figures 1(a) and 1(b)). Imaging showed an enlarged, heterogeneous uterus with multifocal hypodense and cystic foci (Figure 1(c)). She then reported new-onset hematochezia; a CT scan demonstrated a 2.2 cm enhancing, likely a centrally necrotic lesion in the jejunum representing further extramedullary disease (Figure 1(d)).
(a) The (right or left) ovary exhibits stromal hyperplasia that replaces the medulla (hematoxylin and eosin). (b) There is a dense proliferation of oval- to spindle-shaped stromal cells in the ovary exhibiting a vaguely nodular pattern (hematoxylin and eosin). (c) There were a few foci of ovarian stromal hyperthecosis consisting of single cells with small clusters of luteinized cells which have abundant, lightly vacuolated cytoplasm (hematoxylin and eosin). (d) A microscopic granulosa cell tumor was seen in the left ovary which showed the microfollicular cell pattern (Call-Exner bodies; hematoxylin and eosin). (e) Cross-section of the fallopian tube showing focal infiltration by atypical, blastic appearing mononuclear cells (arrow; hematoxylin and eosin). (f) The infiltrating blasts had open chromatin and prominent nucleoli. A few with admixed lymphocytes were also present (arrows; hematoxylin and eosin). (g) Endometrial biopsy showing infiltration by the blasts. Note that the blasts have a moderate amount of eosinophilic cytoplasm (hematoxylin and eosin). (h) Immunohistochemical staining showed that the blasts were CD56 positive (immunoperoxidase).
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Immunoperoxidase staining of paraffin tissue sections showed that these mononuclear cells expressed HLA-DR, CD33, CD56, KP1 (CD68), CD123, and dim focal MPO but were negative for CD34 and CD117, an immunophenotypic profile identical to that of the patient's previously diagnosed AML (Figures 2(a)-2(f)). The cells expressed CD56 and HLA-DR, consistent with MS (Figures 2(g) and 2(h)).