
==== Front
World J Surg Oncol
World J Surg Oncol
World Journal of Surgical Oncology
1477-7819
BioMed Central London

3509
10.1186/s12957-024-03509-z
Case Report
Splenic cyst deroofing complicated with B lymphoma
Justo Iago iagojusto@hotmail.com

12
Jiménez-Romero Carlos 12
Suárez Antonio 12
Vazquez Pablo 12
Revilla Enrique 12
Loinaz Carmelo 12
Bernaldo de Quirós Mercedes 12
1 https://ror.org/02p0gd045 grid.4795.f 0000 0001 2157 7667 Department of Surgery, Faculty of Medicine, Unit of HPB Surgery and Abdominal Organ Transplantation, Complutense University, Madrid, Spain
2 https://ror.org/02p0gd045 grid.4795.f 0000 0001 2157 7667 Department of Pathology, Faculty of Medicine, Unit of Pathology, Complutense University, Madrid, Spain
4 9 2024
4 9 2024
2024
22 23114 3 2024
25 8 2024
© The Author(s) 2024
2024
https://creativecommons.org/licenses/by-nc-nd/4.0/ Open Access This article is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License, which permits any non-commercial use, sharing, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if you modified the licensed material. You do not have permission under this licence to share adapted material derived from this article or parts of it. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by-nc-nd/4.0/.
Background

Splenic cysts are uncommon and very rarely malignant therefore their treatment isn’t standardized. In case of symptomatic cysts different surgical approaches have been suggested. Primary malignant lymphoma of the spleen comprises less than 1% of non-Hodgkin’s lymphomas. To our knowledge, only 203 cases of splenic large B-cell lymphoma (LBCL) have been reported to date and only 2 of them were fibrin-associated splenic cysts.

Case presentation

27-year-old model with a 19 × 13 cm splenic cyst without data of malignancy in the preliminary study and therefore treated with laparoscopic deroofing. After histological diagnosis of LBCL with a fibrin/EBV-associated splenic pseudocyst, the patient received 4 cycles of Rituximab and a laparoscopic splenectomy was performed due to resurgence of the pseudocyst. No evidence of malignancy has been found during follow up (EBV viral load every 3 months during the first year, PET-CT every 6 months during the first year and annual afterwards) performed after the splenectomy.

Discussion and conclusions

The value of tumor markers and radiology for diagnosis of splenic cysts is put into question.

Only 60 cases of Fibrin-associated LBCL (FA-LBCL) have been described in the literature therefore there are no treatment guidelines for them even though surgery together with systemic treatment has been the prevalent route with good results in most cases.

Supplementary Information

The online version contains supplementary material available at 10.1186/s12957-024-03509-z.

Keywords

Splenic cyst
Splenic cell-B lymphoma
Fibrin associated large B-cell lymphomas
issue-copyright-statement© BioMed Central Ltd., part of Springer Nature 2024
==== Body
pmcIntroduction

Splenic cysts are uncommon entities (0.076% in a single study of clinical autopsies performed in California [1]) and are primarily of parasitic origin. Non-parasitic cysts represent only 10% of known cases [2] being for the most part epidermic cysts.

Given their rarity, the management of primary splenic cysts is based entirely on case series and expert opinion. In patients with cysts that are symptomatic at presentation, expert consensus supports different surgical approaches: complete splenectomy [3], cyst-deroofing [4], sclerosis [5] or ¨watch and wait¨ [6]. ﻿The management of large (≥ 5 cm) asymptomatic cysts remains controversial as there is significant lack of evidence to set treatment guidelines.

As previously mentioned, the main cause of true splenic cysts is parasitic infection (Taenia echinococcus and E. granulosus most frequently) even though there are some congenital cases predominantly in children; whereas pseudocysts (which do not have epithelial lining) are primarily posttraumatic [7]. Due to the similarities between cysts and pseudocysts the classification is often blurred. Because of how uncommon they are very little has been published on malignancy related-cysts [8, 9]. Moreover, primary malignant lymphoma of the spleen is also an unusual disease, comprising less than 1% of non-Hodgkin’s lymphomas most of which have a histological diagnosis small B-cell lymphoma. Splenic LBCL are consequently anecdotal.

To our knowledge, only 203 cases of splenic LBCL have been reported to date and only 2 of them were Fibrin Associated Large B-cell lymphomas (FA-LBCL) in splenic cysts [10]. There is a variant of diffuse large B-cell lymphoma associated with chronic cavitary inflammation; there are also cases described in relation to knee-prosthetic replacements, mammary implants, cardiac myxomas and thrombosis [11].

Case presentation

We present the case of a 27-year-old model who presented with abdominal pain and distension, and a visible asymmetric mass in her left hypochondrium. A CT-scan was ordered which showed a 19 × 13 cm splenic cyst. There were no significant analytical findings and serology for toxoplasma, parasitosis, EBVtuberculosis and leishmania was negative. A diagnostic puncture was ordered and the clear liquid content of the cyst was sent for analysis with negative tumor markers and cytology compatible with a splenic pseudocyst. There was no data of malignancy. Due to the large size of the cyst, the presence of symptoms and the interference with our patient´s job surgery was indicated. In absence of suspicion of neoplasia and in an attempt to be as conservative as possible a laparoscopic fenestration of the cyst was performed with aspiration of 3500 cc of turbulent liquid. The histological study of the pseudocapsule was diagnostic for diffuse large B-cell lymphoma developed within a splenic pseudocyst associated with fibrin and EBV with a viral load in serum of over 15 million copies. The immunohistochemistry was positive for CD20, PAX5 and CD79 alpha, expressing CD30 y MUM1 (Fig. 1).Fig. 1 A Small magnification (2x) displays the entire width of the pseudocyst’s capsule. The fibrin deposit can be observed on the top half of the image. B H&E stain 40 × showing groups of large atypical lymphoid cells, high nucleus-cytoplasm ratio, irregular nuclear contour, dense chromatin and clear cytoplasm. C in situ hybridization of the Epstein-Barr virus (EBER) showing an intense and diffuse nuclear staining in all atypical cells

Given these findings a full body PET-scan was performed in which aside from the splenic tumor a parasternal adenopathy was found. Both lesions had pathological metabolic uptake. The case was then presented before a multidisciplinary committee and the patient received hematological treatment with 4 cycles of Rituximab. A new PET-scan was repeated after treatment with single uptake in the pseudocyst which seemed to have closed up again. The EBV load was at this time undetectable. We did not perform anti-EBV antibody titers, like VCA-IgM, VCA-IgG, EADR-IgG, EBNA, because our laboratory is not able to do those tests.

In view of the persistence of the pseudocyst despite previous complete fenestration, radical splenectomy was indicated by the committee. The recovery after surgery was uneventful and the patient was discharged on the second day postoperative.

The histological study of the spleen showed no residue of lymphoma or EBV infection. The patient evolved favorably with no evidence of resurgence of malignancy in postoperative follow ups (EBV viral load every 3 months and PET-CT every 6 months during the first year and annual CT afterwards) almost 2 years after the second procedure.

Discussion and conclusions

We present an exceptionally rare case, as splenic cystic fenestration is an infrequent procedure given the extremely low incidence of splenic cysts and even more uncommon are cavitary lymphomas which is why there is no standardized treatment.

The value of tumor markers in the biopsy of splenic cysts is put into question since it can be elevated in non-neoplastic growths [4] making it difficult to ascertain the appropriate cut-off for low risk of malignancy. Radiology is sometimes insufficient in the diagnosis of tumors within growths with chronic inflammation.

FA-LBCL, as an EBV-related tumor has been acknowledged as a neoplasia in the 5th edition of WHO hematological tumours [12]. It appears to be a growth induced by a state of regional immunosuppression [13]. Only 60 cases have been described in the literature, mainly in relation to knee-prosthetic replacements, mammary implants [14] and cardiac myxomas [15] as mentioned above. No such circumstances were present in our patient.

More recently FA-LBLC have been described in other locations: adrenal glands (4), kidneys (3), spleen (2) [16], gastrointestinal tract [17] and other miscellaneous sites [18].

The other 2 cases of lymphoma within a splenic cyst published were: an accidental finding in a 29-year-old male with a painful 18 cm-cyst treated with splenectomy and subsequent systemic therapy with 4 cycles of Rituximab [13] and a 37-year-old woman with an incidental finding of a 9 cm cyst during a laparoscopy for bariatric surgery. In the latter case a splenectomy was performed followed by 3 cycles of CHOP without evidence of resurgence in either patient in the following 32 months.

The ideal treatment for splenic cell-B lymphoma is unclear since there are to date no randomized studies for such a rare neoplasia [19]. The current consensus is splenectomy with rituximab in monotherapy or combined with other chemotherapics. In our case considering the patient´s age and the absence of comorbidities a complete splenectomy was performed even though in various articles the absence of detectable EBV load allows for monitoring without surgery. It is generally considered to be an indolent disease, however in most series with short follow-ups surgery together with chemo/immunotherapy was indicated. Persistent disease, recurrences or death due to the disease were reported in 4 patients with EBV positive large cells lymphoma, 2 of them due to thrombosis cardiovascular prosthetic graft thrombosis [16].

No patient with pseudocyst-associated FA LBCL showed any evidence of disease during follow-up period after resection [10].

Supplementary Information

Supplementary Material 1.

Abbreviations

CHOP Ciclofosfamida, clorhidrato de doxorrubicina (Hidroxidaunorrubicina), sulfato de vincristina (Oncovin) y prednisona

CT Computed tomography

EBV Epstein-Barr Virus

FA-LBCL Fibrin Associated Large B-cell lymphomas

LBCL Large B-cell lymphomas

Acknowledgements

Not applicable.

Authors’ contributions

Carlos Jiménez-Romero and Iago Justo participated in the data analysis, research design and in the writing of the article. Mercedes Bernaldo de Quirós, Enrique Revilla and Antonio Suárez, participated in the performance of the research. Pablo Vázquez and Carmelo Loinaz participated in the data analysis.

Funding

There research received no external funding.

Availability of data and materials

Datasets used and analyzed during the current study are available from the corresponding author on reasonable request.

Data availability

No datasets were generated or analysed during the current study.

Declarations

Ethics approval and consent to participate

Written informed consent was obtained from the patient prior to submitting the case report and approved by the Hospital Universitario 12 de Octubre Ethics Committee.

Competing interests

The authors declare no competing interests.

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
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