Multiple disciplinary team management of rare primary splenic malignancy: Two case reports

Abstract

Background: Malignant splenic tumors are rare but fatal, presenting a challenge in diagnosis and management involving hematology, oncology, and general surgery. By contrast, diagnosing and treating other common malignant tumors (such as lung and gastrointestinal cancer) offers multiple strategies for chemotherapy, radiotherapy, targeted therapy, and immunotherapy with the prospect of a cure. With various specialists involved in clinical multiple disciplinary team (MDT) discussion, personal bias can be minimized. It can also ignite important discussion which can benefit not only one patient but many patients.

Case summary: Here, we report on the MDT diagnosis and management of the malignant splenic tumors littoral cell angiosarcoma and histiocytic sarcoma. Although only two cases of rare primary splenic malignancy are presented, MDT is a novel means of rare disease treatment.

Conclusion: To benefit patients, imaging analysis, safe operation, precise pathology examination, and individualized therapeutic treatment strategies are required. The involvement of various specialists in a clinical MDT discussion minimizes personal bias and can create important ideas to benefit all patients.

Keywords: Case report; Histiocytic sarcoma; Littoral cell angiosarcoma; Multiple disciplinary team; Splenic malignancy.

Figure 1

Abdominal computed tomography angiograph scan of Case 2. A: (arterial phase) and B (venous phase): In transverse section, A pre-operative computed tomography (CT) scan revealed splenomegaly, and a tumor (6.0 cm × 5.7 cm in size) with a CT value 48 HU showing gradual enhancement; C (arterial phase) and D (venous phase): The splenic tumor is shown in the coronal phase. White arrow indicates the tumor.

Figure 2

HE and immunohistochemical characteristics of Case 1. A: The littoral cell angiosarcoma contains perivascular sinus-like heterocysts with dark nuclei and multiple mitotic phases on HE staining; B and C: Immunohistochemical phenotype analysis showed that the tumor cells were CD31 positive, while CD68 was focally positive; D and E: Furthermore, typical endothelial markers CD34 and ERG were positive and expressed in perivascular cells in littoral cell angiosarcoma; F: The Ki-67 index was 5%-10% (black arrow).

Figure 3

HE and immunohistochemical characteristics of Case 2. A: The tumor in Case 2 contained numerous large cells with abundant blue cytoplasm with binucleated and trinucleated cells, which is coincident with the characteristics of histiocytic sarcoma; B and C: Immunohistochemical phenotype analysis showed that tumor cells were CD31 positive, while CD68 was generally positive; D and E: CD34 and ERG were only positively expressed in vascular endothelial cells; F: The Ki-67 index was 15%-20%.