Primary Carcinosarcoma of the Spleen: Case Report of a Rare Tumor and Review of The Literature

Abstract

Carcinosarcoma of extragenital organs is rare. In this article, a case of primary carcinosarcoma of the spleen, which presented as painful splenomegaly is reported. To the best of our knowledge, this is the second reported case of primary splenic carcinosarcoma in English literature. The pathogenesis of these tumors is incompletely understood. No specific treatment guidelines exist for these aggressive tumors, surgery being the mainstay of treatment. The prognosis remains poor regardless of the adjuvant therapy used.

Primary splenic carcinosarcoma with local invasion of chest wall: a rare case

“…They occurred in many anatomical locations such as the female genital tract (Dave et al, 2011;Kanthan and Senger, 2011), pelvic peritoneum (Ko et al, 2005), breast (Cornette et al, 2005), stomach (Iezzoni and Mills, 1993), colon (Isimbaldi et al, 1996), pancreas (Darvishian et al, 2002;Chmiel et al, 2005), head and neck, respiratory system, hepatobiliary system, thyroid, thymus, and skin. A malignant mass arising within the spleen is most commonly of a lymphoreticular origin (Kochar et al, 2009). The finding of carcinosarcoma in the spleen is very rare.…”

“…Many preoperative and/or postoperative examinations, such as physical examination, abdominal ultrasound, CT scan, and endoscopic examination of the gastrointestinal tract, have to be conducted to exclude the metastatic neoplasms prior to the diagnosis of primary splenic tumor (Kochar et al, 2009). In our limited experience, PET/CT could be helpful to the above procedures.…”

“…We are more inclined to believe that the neoplasm in this case probably arose directly from the spleen, in the absence of evidence to support metastasis from other sites. So far, the survival time of splenic carcinosarcoma patients post operation ranged from 3 to 12 months (Westra et al, 1994;Rao et al, 2007;Kochar et al, 2009;Joy et al, 2012). This may be because of the rapid spread and highly invasive nature of these lesions (Rao et al, 2007).…”

“…It is generally agreed that complete macroscopic excision of the malignancy may provide the best chance of survival (Kochar et al, 2009). Postoperatively, adjuvant treatments, such as chemotherapy and radiotherapy, should be under consideration to further kill the tumor cells and improve the prognosis of patients.…”

“…Rao et al (2007) reported a case of splenic carcinosarcoma of a male, which was characterized by the presence of an osteosarcomatous element and raised β human chorionic gonadotropin (β-hCG) levels. Kochar et al (2009) presented a Caucasian man with a high-grade malignant pleomorphic tumor with areas of necrosis, and this case revealed osteosarcomatous differentiation and a spindle and epithelioid morphology. Most recently, Joy et al (2012) reported another case of primary splenic carcinosarcoma.…”