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Primary splenic epithelioid mesothelioma: A rare occurrence
1 MD, Senior Resident, Madurai Medical College, Madurai, India
2 Medical Student, UTMB, Galveston, Texas, USA
3 MD, Senior Resident, Tanjore Medical College, Tanjore, India
4 MD, 1912 Aberdeen Ave, Lubbock, Texas 79407, USA
Address correspondence to:
Seshadri Thirumala
MD, 1912 Aberdeen Ave, Lubbock, Texas 79407,
USA
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Article ID: 100031Z11NJ2019
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Jayakumar N, Manoharan D, Thirumala V, Thirumala S. Primary splenic epithelioid mesothelioma: A rare occurrence. J Case Rep Images Pathol 2019;5:100031Z11NJ2019.ABSTRACT
No Abstract
Keywords: Epithelioid mesothelioma, Spleen
Case Report
A 63-year-old multiple sclerosis patient presented to our institution with a history of sudden abdominal pain of two weeks duration. The pain was predominantly localized to the left upper quadrant radiating to the back. On examination, mild tenderness of the abdomen was noted. Peripheral lymphadenopathy was absent. Computed tomography (CT) scan of abdomen and pelvis showed moderate to massive splenomegaly measuring 15 cm in greatest dimension below the costal line, with a large heterogeneous mass measuring 11 × 7 cm. Positron emission tomography (PET) CT revealed intense fluorodeoxyglucose (FDG) accumulation which was worrisome for lymphoproliferative disorder. No other abnormal FDG uptake elsewhere in the body was noted. Robotic-assisted splenectomy was performed and the specimen was sent to Pathology Laboratory for evaluation including for lymphoma work-up. Grossly, the spleen weighed 754 g and measured 21 × 10.5 × 8 cm and on cut section showed multiple tan nodules ranging in size from 0.1 × 0.1 cm to 12 × 9 × 5.5 cm (Figure 1A). Sections from the attached adipose tissue revealed multiple, tiny matted nodes.
Histologic evaluation showed a malignant predominantly epithelioid tumor (Figure 1B). Focal papillary and clear cell features were present with extensive areas of necrosis and vascular invasion. The tumor cells were diffusely positive for CK 7, mesothelioma markers including D2-40 (Figure 1C), Calretinin (Figure 1D), WT1. All other antibodies tested against including estrogen receptor (ER), progesterone receptor (PR), PAX-8, TTF-1, SOX10, CD34, CD31, S100, ETS-related gene (ERG), CDX2 and Inhibin turned were negative. Two out of eight lymph nodes showed metastatic deposits. Based on morphology and immunohistochemical features a diagnosis of splenic epithelioid mesothelioma was rendered.
Discussion
Malignant mesothelioma arises from the mesothelial surfaces of pleura, peritoneum, pericardium, and tunica vaginalis. History of occupational asbestos exposure can be elicited in most cases.
Primary malignant mesothelioma in solid organs is unusual and only handful of cases involving lung, gonads, liver, spleen, and pancreas have been reported in English language literature [1]. These occur as localized malignant mesotheliomas in the absence of pleural, peritoneal, or pericardial involvement. Several theories have been put forth to explain the origin of these tumors in solid organs including from an invaginated capsular epithelium [2],[3] or a preexisting inclusion cyst or transition of native epithelial cells. To the best of our knowledge, this is the second case of splenic malignant mesothelioma reported in the literature. Differential diagnosis for splenic mesothelioma includes a wide variety of benign and malignant etiologies including localized mesothelioma, lymphomas, epithelioid angiosarcoma, and metastases [3]. Careful histologic evaluation with a panel of mesothelial markers will help arrive at the correct diagnosis.
Conclusion
In conclusion, we report the second case of primary splenic mesothelioma in the absence of serosal involvement.
REFERENCES
1.
Liu YC, Kuo YL, Yu CP, et al. Primary malignant mesothelioma of the greater omentum: Report of a case. Surg Today 2004;34(9):780–3. [CrossRef]
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Del Sordo R, Bellezza G, Colella R, Mameli MG, Giansanti M, Cavaliere A. Epithelial cyst of the spleen with foci of ectopic liver. J Pediatr Surg 2011;46(1):e21–3. [CrossRef]
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Giansanti M, Bellezza G, Guerriero A, Pireddu A, Sidoni A. Localized intrasplenic mesothelioma: A case report. Int J Surg Pathol 2014;22(5):451–55. [CrossRef]
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SUPPORTING INFORMATION
Author Contributions
Niveditha Jayakumar - Conception of the work, Design of the work, Drafting the work, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Dhivya Manoharan - Conception of the work, Design of the work, Drafting the work, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Vani Thirumala - Conception of the work, Design of the work, Drafting the work, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Seshadri Thirumala - Conception of the work, Design of the work, Drafting the work, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Guarantor of SubmissionThe corresponding author is the guarantor of submission.
Source of SupportNone
Consent StatementWritten informed consent was obtained from the patient for publication of this article.
Data AvailabilityAll relevant data are within the paper and its Supporting Information files.
Conflict of InterestAuthors declare no conflict of interest.
Copyright© 2019 Niveditha Jayakumar et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.
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