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CASE REPORT
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Unusual case of follicular lymphoma presenting as solitary painless scalp swelling: Case report and review of literature


 Department of Neurosurgery, Ruby Hall Clinic, Pune, Maharashtra, India

Date of Submission23-Apr-2021
Date of Decision05-Jul-2021
Date of Acceptance28-Oct-2021

Correspondence Address:
Rahul Sharma,
Department of Neurosurgery, Associate Consultant, Ruby Hall Clinic, Pune, Maharashtra
India
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/mjdrdypu.mjdrdypu_288_21

  Abstract 


Follicular lymphoma (FL) is the second most frequent non-Hodgkin lymphoma accounting for about 10%–20% of all lymphomas. FL usually presented with peripheral adenopathy involving cervical, inguinal, and femoral regions. FL presenting as a scalp swelling without involvement of underlying cerebral parenchyma or systemic manifestation in a nonimmunocompromised patient is an extremely rare medical condition requiring aggressive treatment and neurosurgical intervention. We report the case of a 33-year-old male presenting with progressively increasing painless large left-sided frontal scalp swelling. The patient underwent near-total excision of lesion. Histopathology report suggested FL. We report this case for discussion regarding pathophysiology, diagnosis, and appropriate clinical management.

Keywords: Follicular lymphoma(fl), non hodgkin lymphoma(nhl), scalp swelling



How to cite this URL:
Sharma R, Katkar A, Bhanage A. Unusual case of follicular lymphoma presenting as solitary painless scalp swelling: Case report and review of literature. Med J DY Patil Vidyapeeth [Epub ahead of print] [cited 2022 Dec 6]. Available from: https://www.mjdrdypv.org/preprintarticle.asp?id=332582




  Introduction Top


Follicular lymphoma (FL) is the second most frequent non Hodgkin lymphoma accounting for about 10%–20% of all lymphomas. FL usually presented with peripheral adenopathy involving cervical, inguinal, and femoral regions. FL presenting as a scalp swelling without involvement of underlying cerebral parenchyma or systemic manifestation in a nonimmunocompromised patient is an extremely rare medical condition requiring aggressive treatment and neurosurgical intervention. This report present a case of FL of scalp in nonimmunocompromised individual and reviews of literature.Moreover ,we use the case to illustrate the importance of early detection and appropriate clinical management.


  Case Report Top


A 33-year-old male working as a computer sales person presented with progressively increasing painless swelling in the left frontal region for 6 months [Figure 1]. On local examination, a 7 cm × 3 cm hard, noncompressible swelling was seen in the left frontal scalp.
Figure 1: Left frontal scalp swelling

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The patient had no neurological signs. Computed tomography (CT) scan of the brain showed soft-tissue swelling in the left frontal region 6.9 cm × 1.2 cm × 2.5 cm with underlying sclerotic left frontal bone [Figure 2]. Magnetic resonance imaging brain shows altered marrow signals in the frontal bone with adjacent heterogeneously enhancing soft tissue and underlying dural thickening. Preoperatively, the diagnosis of lymphoma was uncertain because radiological confirmation was in favor of osteomyelitis.
Figure 2: Computed tomography scan revealing soft-tissue swelling in the left frontal region, magnetic resonance imaging T2-weighted image axial and sagittal showing altered marrow signals in the frontal bone and soft-tissue swelling which was hyperintense on T2-weighted image, and magnetic resonance imaging T1-weighted image contrast showing heterogeneously enhancing soft tissue measuring 12 mm in width, causing bulge in the frontal scalp

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The patient underwent left frontal craniectomy with gross total excision of the soft-tissue swelling. Underlying bone was hyperostotic and dura was thickened. Hyperostotic bone was excised and dural surface was cauterized. The craniectomy defect was reconstructed using titanium mesh and titanium screws. Histopathology report of soft tissue showed lymphoid cells with abundant lymphoid follicles. Cells were small-to-medium size with scanty cytoplasm. On immunohistochemistry examination, cells were positive for CD3, CD20, CD10, BCL2, and BCL6. These findings were consistent with follicular lymphoma Grade 2 [Figure 3].
Figure 3: (a and d) Histopathology staining showing follicular pattern, centrocyte, and centroblast, (b, c, and e) Immunohistochemistry showing cell positive for BCL2 positive (b), CD10 (c), CD20 (e)

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Positron emission tomography (PET) CT scan showed fluorodeoxyglucose-avid soft-tissue density in the left forehead region, likely residual disease. No metabolically active disease seen elsewhere in the body [Figure 4]. The patient underwent radiotherapy 30.6 Gy in 17 fractions, followed by boost to a dose of 5.4 Gy in 3 fractions. As it was a Stage 1 follicular lymphoma, chemotherapy was deferred. Follow-up PET scan after the radiotherapy showed no new active lesion and good response to the therapy. Currently, the patient is regularly monitored on an outpatient department basis.
Figure 4: Positron emission tomography–computed tomography scan showing no focal fluorodeoxyglucose-avid skeletal lesions

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  Discussion Top


Non-Hodgkin lymphomas (NHLs) occur more frequently in patients with acquired immunodeficiency syndrome and represent 3%–4% of neoplasm in general population. Primary involvement of bone by NHL occurs in 3%–4% of the cases[1] and usually involves femur, tibia, pelvis, spine, and mandible.[2] Primary lymphoma involving scalp without any systemic involvement is extremely rare[3] in a nonimmunocompromised patient. A primary lymphoma of the scalp is defined as a single mass lesion without any evidence of systemic involvement or other sites. The initial symptoms and signs include a painless scalp lump (90%), headache (30%), focal neurological deficit (20%), and seizures (10%). In our case, painless scalp swelling was the only manifestation without any neurological deficit. These lesions are reported in older patients with a mean age of 60.5 years. Our patient was seronegative and presented at a young age as compared to other reported cases.[2],[4]

After the diagnosis of cranial vault/scalp lymphoma was confirmed, PET scan was done to rule out any systemic involvement. Our patient did not have any evidence of systemic involvement and lesion was localized to the scalp/vault region. Scalp lymphoma is effectively treated by surgery and radiotherapy with good clinical outcome in most cases. Due to the paucity of cases in literature, the optimal management of primary bone lymphoma remains uncertain. However, surgical removal, followed by radiotherapy and chemotherapy,can be recommended.[2] Our case was managed by wide excision of soft tissue swelling along with involved bone followed by radiotherapy with good clinical outcome. However, chemotherapy was deferred as it was limited stage (I–II) follicular lymphoma.[5]


  Conclusion Top


Primary follicular lymphoma of the scalp without systemic involvement is effectively treated by surgery followed by radiotherapy and chemotherapy. However, adjuvant chemotherapy treatment depends upon stages of follicular lymphoma.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Curty B, Kernan J, Favre J. Malignant Non Hodgkins lymphoma of the cranial vault: A case report. Br J Neurosurg 1997;11:433-6.  Back to cited text no. 1
    
2.
El Asri AC, Akhaddar A, Baallal H, Boulahroud O, Mandour C, Chahdi H, et al. Primary lymphoma of the cranial vault: Case report and a systematic review of the literature. Acta Neurochir (Wien) 2012;154:257-65.  Back to cited text no. 2
    
3.
Fukushima Y, Oka H, Utsuki S, Nakahara K, Fujii K. Primary malignant lymphoma of the cranial vault. Acta Neurochir (Wien) 2007;149:601-4.  Back to cited text no. 3
    
4.
Nwafor D, Radwan W, Lucke-Wold B, Underwood W, Gyure K, Marsh R. Follicular lymphoma presenting as scalp mass deformity: Case Report and Review of the literature. Biomed Res Clin Pract. 2018;3:10.15761/BRCP.1000155. doi: 10.15761/BRCP.1000155. Epub 2018 Feb 24. PMID: 30057944; PMCID: PMC6059655.  Back to cited text no. 4
    
5.
Luminari S, Bellei M, Biasoli I, Federico M. Follicular lymphoma treatment and prognostic factors. Rev Bras Hematol Hemoter 2012;34:54-9.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

 
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