Skip to main content

Main menu

  • Latest content
    • Latest content
  • Archive
  • About the journal
    • About the journal
    • Editorial board
    • Information for authors
    • FAQs
    • Thank you to our reviewers
    • The American Association for the Surgery of Trauma
  • Submit a paper
    • Online submission site
    • Information for authors
  • Email alerts
    • Email alerts
  • Help
    • Contact us
    • Feedback form
    • Reprints
    • Permissions
    • Advertising
  • BMJ Journals

User menu

  • Login

Search

  • Advanced search
  • BMJ Journals
  • Login
  • Facebook
  • Twitter
TSACO

Advanced Search

  • Latest content
    • Latest content
  • Archive
  • About the journal
    • About the journal
    • Editorial board
    • Information for authors
    • FAQs
    • Thank you to our reviewers
    • The American Association for the Surgery of Trauma
  • Submit a paper
    • Online submission site
    • Information for authors
  • Email alerts
    • Email alerts
  • Help
    • Contact us
    • Feedback form
    • Reprints
    • Permissions
    • Advertising
Open Access

Surgical solution for a paraneoplastic neurodegenerative disorder

Huda Muhammad, Daniel G Strayve, Raja R Narayan, Douglas W Blayney, Daniel Delitto
DOI: 10.1136/tsaco-2022-000928 Published 2 May 2022
Huda Muhammad
1Section of Surgical Oncology, Department of Surgery, Stanford University School of Medicine, Stanford, California, USA
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Huda Muhammad
Daniel G Strayve
2Department of Surgery, Touro College and University System, Vallejo, California, USA
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Raja R Narayan
1Section of Surgical Oncology, Department of Surgery, Stanford University School of Medicine, Stanford, California, USA
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Douglas W Blayney
3Division of Oncology, Department of Medicine, Stanford University School of Medicine, Stanford, California, USA
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Daniel Delitto
1Section of Surgical Oncology, Department of Surgery, Stanford University School of Medicine, Stanford, California, USA
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • Article
  • Figures & Data
  • eLetters
  • Info & Metrics
  • PDF
Loading

Surgical dilemma

A 58-year-old man with a history of obesity status post Roux-en-Y gastric bypass and chronic lymphocytic leukemia (CLL) with aggressive features in complete remission 10 months after bendamustine and rituximab chemotherapy presented with neurological decline. He previously was a high-functioning information technology expert who 3 weeks earlier began having difficulty with fine movement when using his mobile device. He presented at a local hospital with new-onset dysarthria and word-finding difficulties, prompting admission. He underwent extensive neurological workup, which was negative. He was given an empiric trial of steroids due to suspicion of a neurological paraneoplastic syndrome and had minimal improvement. Cross-sectional imaging of the chest, abdomen, and pelvis showed splenomegaly, with no other lesions or lymphadenopathy. He was transferred to our referral center for further management.

He presented with white blood cell count of 3.1, hemoglobin of 11.5 g/L, and platelet count of 53,000/uL. An fluorodeoxyglucose (FDG)-positron emission tomography (PET) scan was notable only for high metabolic activity in the superior pole of the spleen, concerning for recurrent lymphoma (figure 1). We suspected transformation of CLL into diffuse large B cell lymphoma (DLBCL) (ie, Richter’s syndrome) with accompanying paraneoplastic manifestations. On initial evaluation by the surgical team, he was alert and oriented but had expressive aphasia and word-finding difficulties. On re-evaluation in the afternoon, he was not speaking and no longer following commands. His platelet count was low and minimally responsive to transfusion.

Figure 1
  • Download figure
  • Open in new tab
  • Download powerpoint
Figure 1

FDG-PET imaging reveals an avid lesion in the anterior superior pole of the spleen, concerning for recurrent malignant disease. There is no evidence of other lymphadenopathy and the bone marrow did not show abnormal uptake in this or other images.

What would you do?

  1. Total splenectomy

  2. Partial splenectomy

  3. Bone marrow biopsy

  4. Continue steroids and attempt to initiate chemotherapy

What we did and why

A. Total splenectomy.

In consultation with the oncology team, the patient was taken for a laparoscopic hand-assisted splenectomy. Additional steroids were given the evening prior to surgery and platelets were transfused in the operating room. Given concerns for extranodal lymphoma, the spleen was not morselized but instead removed intact through the hand port to preserve the architecture for pathological study (figure 2).

Figure 2
  • Download figure
  • Open in new tab
  • Download powerpoint
Figure 2

Surgical specimen after laparoscopic hand-assisted splenectomy.

The decision to operate was controversial in light of his severely deconditioned state, steroid requirement, and thrombocytopenia. His neurological function at the time of surgery could certainly be interpreted as a poor prognostic sign, suggesting palliative measures may have been more appropriate. However, given the rapidity of his decline and high-functioning status just weeks prior to his admission, we favored a potentially reversible process. Partial splenectomy is rarely warranted in an adult patient due to postoperative bleeding risks and could not have been safely performed for this lesion. The diagnostic yield of bone marrow biopsy is low in this situation and the associated delay could lead to irreversible neurological decline. Although steroid administration was initially associated with some improvement, rapid neurological decline ensued and chemotherapy was unlikely to be tolerated in his near comatose state.

He tolerated the operation well without complications. Postoperatively, he experienced swift recovery of neurological function and resolution of thrombocytopenia. On postoperative day 1, he was following commands and ambulating. By postoperative day 7, he exhibited near-complete neurological and hematological recovery. His marked recovery supported the assertion that his decline was related to a paraneoplastic syndrome from his hematological malignancy. Histopathology of the splenic mass confirmed DLBCL.

This patient course highlights two important phenomena in the progression of lymphoproliferative disorders. First, Richter’s syndrome is a broad term describing transformation of low-grade lymphoproliferative disorders to more aggressive lymphomas, the most common being CLL to DLBCL (figure 3). Richter’s transformation is multifactorial, associated with genetic aberrations, immunosuppression, and certain therapies used in the treatment of CLL, namely purine analogs and alkylating agents. When such transformation takes place, patients may rapidly decline, especially in cases of clonal expansion from the primary malignancy with early signs of disease, manifesting as lymphadenopathy, extranodal spread, or multiorgan failure. Second, the initial presentation of this patient was a rapid neurological decline—a paraneoplastic syndrome indicative of specific targeting of the central nervous system (CNS). Paraneoplastic syndromes of the CNS have previously been described in cases of aggressive lymphomas, notably DLBCL, where patients develop neuropathies and cerebellar degeneration due to overproduction of cytokines or antibodies, produced by malignant B cells, targeting CNS antigens. Paraneoplastic syndromes are exceedingly rare, with <1% incidence in solid tumors and even less frequent in lymphoma. Interestingly, the extranodal tumor burden in this patient was directly related to the severity of the paraneoplastic syndrome, with resection resulting in rapid resolution.

Figure 3
  • Download figure
  • Open in new tab
  • Download powerpoint
Figure 3

Natural history of Richter’s transformation from chronic lymphocytic leukemia (CLL) to diffuse large B cell lymphoma (DLBCL).

Unless there is a mechanical complication associated with lymphoma, such as an obstruction or perforation, surgical resection is not the treatment of choice. Instead, chemotherapy is the optimal treatment for this otherwise systemic disease. However, in this case, there was a single focus of malignancy that could be resected without undue morbidity. His mental status then improved to the point that he could begin definitive treatment with chemotherapy. This case further highlights the importance of a multidisciplinary management of complex oncological cases with atypical presentations.

Ethics statements

Patient consent for publication

Not required.

Footnotes

  • Contributors HM and DGS were responsible for literature and case review, article drafting, and article revisions. RRN was responsible for project conception, case review, and article revisions. DWB was responsible for case review and article revisions. DD was responsible for project conception, case review, and article revisions. All authors approve of the final article version.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; internally peer reviewed.

http://creativecommons.org/licenses/by-nc/4.0/

This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/.

PreviousNext
Back to top
Email

Thank you for your interest in spreading the word on TSACO.

NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. We do not capture any email address.

Enter multiple addresses on separate lines or separate them with commas.
Surgical solution for a paraneoplastic neurodegenerative disorder
(Your Name) has sent you a message from TSACO
(Your Name) thought you would like to see the TSACO web site.
CAPTCHA
This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.
Print
Alerts
Sign In to Email Alerts with your Email Address
Citation Tools
Surgical solution for a paraneoplastic neurodegenerative disorder
Huda Muhammad, Daniel G Strayve, Raja R Narayan, Douglas W Blayney, Daniel Delitto
Trauma Surg Acute Care Open May 2022, 7 (1) e000928; DOI: 10.1136/tsaco-2022-000928

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero
Cite This
  • APA
  • Chicago
  • Endnote
  • MLA
Loading
Surgical solution for a paraneoplastic neurodegenerative disorder
Huda Muhammad, Daniel G Strayve, Raja R Narayan, Douglas W Blayney, Daniel Delitto
Trauma Surg Acute Care Open May 2022, 7 (1) e000928; DOI: 10.1136/tsaco-2022-000928
Download PDF

Share
Surgical solution for a paraneoplastic neurodegenerative disorder
Huda Muhammad, Daniel G Strayve, Raja R Narayan, Douglas W Blayney, Daniel Delitto
Trauma Surgery & Acute Care Open May 2022, 7 (1) e000928; DOI: 10.1136/tsaco-2022-000928
del.icio.us logo Digg logo Reddit logo Twitter logo CiteULike logo Facebook logo Google logo Mendeley logo
Respond to this article
  • Tweet Widget
  • Facebook Like
  • Google Plus One
  • Article
    • Surgical dilemma
    • What would you do?
    • What we did and why
    • Ethics statements
    • Footnotes
  • Figures & Data
  • eLetters
  • Info & Metrics
  • PDF

Related Articles

Cited By...

More in this TOC Section

  • Management of an internal hernia through the foramen of Winslow
  • Bouveret syndrome: approaching the obstructive duodenal gallstone
  • Ventriculoperitoneal shunt migration into an inguinal hernia with subsequent laparoscopic reduction and hernia repair
Show more Challenges in trauma and acute care surgery

Similar Articles

 
 

CONTENT

  • Latest content
  • Archive
  • eLetters
  • Sign up for email alerts
  • RSS

JOURNAL

  • About the journal
  • Editorial board
  • Thank you to our reviewers
  • The American Association for the Surgery of Trauma

AUTHORS

  • Information for authors
  • Submit a paper
  • Track your article
  • Open Access at BMJ

HELP

  • Contact us
  • Reprints
  • Permissions
  • Advertising
  • Feedback form

©Copyright 2022 The American Association for the Surgery of Trauma