Extramedullary hematopoiesis secondary to malignant solid tumors: a case report and literature review

Abstract

Extramedullary hematopoiesis (EMH) usually occurs in hematological disease, but more rarely develops in cases of malignant solid tumors. Due to its features on computed tomography (CT) and magnetic resonance imaging (MRI) that are atypical, EMH in tumor patients might easily be misdiagnosed as metastasis leading to the improper TNM staging and inappropriate therapy. Here, we reported the first case of pleural EMH occurring in a patient with esophageal carcinoma whose pleural lesion was first diagnosed as metastasis and confirmed EMH after the needle biopsy. In addition, a retrospective review was conducted by analyzing patients presented with EMH with malignant solid tumors from PubMed and Medline databases. A total of 42 solid tumor patients with EMH were enrolled, and breast cancer was the most common (n=13, 31.0%), followed by renal carcinoma (n=7, 16.7%) and lung cancer (n=6, 14.3%). A wide variety of body sites may be affected by EMH in malignant solid tumor patients, of which the lymph nodes (n=8, 19.0%) and liver (n=7, 16.7%) were the most common, followed by the kidney (n=6, 14.3%). All patients were diagnosed with EMH by excision, biopsy, or autopsy. Treatment strategies for EMH included surgery (n=25, 59.5%), hydroxyurea (n=1, 2.4%), and blood transfusions (n=2, 4.8%); a further 14 patients (33.3%) were subjected to clinical observation without intervention. Of the patients for whom outcome was reported, 10 patients maintained a good performance status (23.8%) and a further six patients died from the malignant tumor. This was the first study to summarize the presentations of EMH in malignant solid tumors, and our findings might provide some useful guidance for clinical practice, especially for treating patients harboring nonresponse lesions during the antitumor treatment.

Keywords: biopsy; cancer; extramedullary hematopoiesis; imaging features; sarcoma.

Figure 1

Changes in CT in the ESCC. Notes: (A and D) the well-circumscribed mass in right pleura (yellow arrows) and thickened esophageal wall (red arrow) prior to treatment, respectively. (B and E) Reduction in the lesions for the esophageal wall (red arrow) and stabilization of the lesion for the right pleura (yellow arrows) after two cycles of TP chemotherapy. (C and F) Esophageal wall (red arrow) thickness decrease and pleural lesion (yellow arrows) size stability after four cycles of TP chemotherapy. Abbreviations: CT, computed tomography; ESCC, esophageal squamous cell carcinoma; TP, cisplatin–docetaxel.

Figure 2

(A) Pathocytology of esophageal tissue revealed squamous cell carcinoma. (B and C) Pathocytology of pleural soft tissue showed a few of lymphocytes, erythropoiesis, megakaryocytes, and degraded cells. Notes: (A and B) H&E staining ×100. Scale bar: 100 µm and (C) H&E staining ×200. Scale bar: 100 µm. Abbreviation: H&E, hematoxylin and eosin.

Figure 3

Immunohistochemical of pleural mass revealed EMH. Notes: Immunohistochemical staining ×200. Scale bar: 200 µm. (A) CD15⁺; (B) CD20⁺; (C) CD34⁺; (D) CD30⁺; (E) CD38⁺; (F) CD68⁺; (G) CD7⁺; (H) Ki67⁺ (80–90%); (I) lysozyme⁺; (J) CK5^-; and (K) CK7^-. Abbreviation: EMH, extramedullary hematopoiesis.

Figure 4

Variations in CT in the ESCC 1 month after finishing all the treatments. Note: (A and B) The stabilization of the right pleural mass (yellow arrows) and decrease of the thickened esophageal wall (red arrow) after the treatment, respectively. Abbreviations: CT, computed tomography; ESCC, esophageal squamous cell carcinoma.

Figure 5

T2W/TSE sagittal MRI image 1 month after the completion of all the treatments, showing no considerable change in the size of the right pleural lesion (yellow arrow). Abbreviations: MRI, magnetic resonance imaging; T2W/TSE, T2 weighted/turbo spin echo.

Figure 6

Process for the diagnosis and treatment of EMH in malignant solid tumors. Abbreviations: CT, computed tomography; EMH, extramedullary hematopoiesis; MRI, magnetic resonance imaging.