A patient with myelodysplastic syndrome (refractory anemia with excess of blasts in transformation, RAEB-T) presented with generalized granulocytic sarcomas involving the skin. The diagnosis was confirmed by skin and bone marrow biopsies. Partial myeloperoxidase deficiency was noted in the circulating polymorphonuclear leukocytes and the more differentiated tumor cells in the granulocytic sarcoma. This observation suggests that such leukocytes may be derived from the abnormal ‘leukemic’ clone.
Paragangliomas occurring primarily in the liver parenchyma are extremely rare. The radiological features mimic hepatocellular carcinoma. Herein, we describe a case with a presurgical diagnosis of hepatocellular carcinoma, which was pathologically confirmed as hepatic paraganglioma after surgery. A 41-year-old woman visited our hospital for evaluation of a hepatic mass detected incidentally in a health examination 7 years previously. Triple-phase computed tomography of the liver showed a homogeneous and strongly arterial enhancing mass, followed by portovenous washout at the posterior segment of the right lobe. In view of the enhancement pattern and highly vascular nature, the hepatocellular carcinoma was included in the differential diagnosis. Anatomical resection of segment VII was carried out. Grossly, the mass was encapsulated and soft. Microscopically, it had a typical “Zellballen pattern.” Immunohistochemical staining for CD56 was positive, and sustentacular cells at the periphery of the tumor cell nests were also demonstrated by S-100 protein. Therefore, the diagnosis of primary paraganglioma of the liver was confirmed. In conclusion, hepatic paraganglioma should be considered in the differential diagnosis in cases of hepatic masses displaying “early enhancement, early washout.”
Teratoma of the ovary is the most frequently encountered germ cell tumor. It usually occurs in young women. Gliomatosis peritonei (GP) is mature neural glial tissue implanted onto the peritoneal surface. We present a case of a mature teratoma accompanied by GP and massive ascites in postmenopausal women. A 54-year-old, G0P0, woman presented in the gynecology outpatient department with abdominal distension for 6 months. Computed tomography scan of the abdomen and pelvis displayed an ovarian mass about 20 cm × 18 cm with peritoneal seeding, ascites, and enlarged paraaortic lymph nodes. A total hysterectomy and bilateral adnexectomy were performed. The pathology showed the left ovary contained a dermoid cyst. The biopsy of the peritoneal nodule displayed glial tissue confirming the diagnosis of GP. The patient remained in good condition 6 months postoperatively. We suggest GP be considered in patients presenting with teratomas and massive ascites. The radiological diagnosis is challenging due to the rarity of GP. Continued follow-up of patients with teratomas and GP is mandatory due to the potential of malignant transformation.
Purpose To report a case of an orbital myeloid sarcoma concurrent with JAK2 mutation myelofibrosis, which rapidly transformed into acute myeloid leukemia upon aggressive treatment. Results A 51-year-old woman had progressive swelling of periorbita for one month. Magnetic resonance imaging demonstrated a well-defined, mild enhanced mass indenting the adjacent right lateral rectus muscle and the globe. Biopsy from anterior orbitotomy revealed an orbital myeloid sarcoma. Bone marrow study showed concurrent myelofibrosis. Although the orbital lesion subsided remarkably under aggressive chemotherapy and radiotherapy, the leukemic transformation was noticed in the third month following the initial presentation. Conclusion This case demonstrated that myeloid sarcoma should be included in the differential diagnosis of orbital diseases, with or without involvement of hematological disorders. Early diagnosis and aggressive treatment as for AML are crucial as the prognosis is usually poor for adult orbital MS.
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