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LETTER TO EDITOR |
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| Ahead of print
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| Aggressive large B cell lymphoma mimicking turban tumor |
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Ashish Singh1, Charanpreet Singh2, Ridhi Sood3, Gaurav Prakash2
1 Department of Internal Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Clinical Hematology and Medical Oncology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
3 Department of Histopathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
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| Date of Submission | 24-Sep-2022 |
| Date of Decision | 08-Nov-2022 |
| Date of Acceptance | 14-Nov-2022 |
| Date of Web Publication | 23-Mar-2023 |
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Dear Editor,
A 42-year-old gentleman presented with a history of progressively increasing and painless scalp swelling over the occipital region for eight months. There were no accompanying neurological or constitutional symptoms. Clinically, he had a large, firm, lobulated, non-tender mass, predominantly in the occipital and temporal region, measuring around 20 × 12 cm. It gave an appearance of a large turban-like tumor [Figure 1]a. | Figure 1: (a) Scalp mass in occipital region ~ 20 × 12 cm (before chemotherapy). (b) CE-MRI brain shows a heterogeneously enhancing extra-axial and extra-cranial mass in bilateral occipital and left temporoparietal region, with max. thickness being 9.8 cm over occiput and no abnormal meningeal or parenchymal enhancement (before chemotherapy). (c) Near complete disappearance of scalp mass (after chemotherapy). (d) CE-CT brain shows a significant reduction in size of scalp mass (2.7 cm): nCR (after chemotherapy)
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Contrast-enhanced magnetic resonance imaging (CE-MRI) of the brain revealed a heterogeneously enhancing extra-axial and extra-cranial mass in the bilateral occipital and left temporoparietal region, with no abnormal meningeal or parenchymal enhancement [Figure 1]b. There was no evidence of systemic lymphoma elsewhere in the body. During the course of evaluation for lymphoma, the patient tested positive for HIV, with a baseline CD4 count of 180 cells/μL. A core biopsy from the scalp mass showed sheets of atypical lymphoid cells in a collagenous background [Figure 2]a. On immunohistochemistry, these atypical lymphoid cells were positive for CD10, CD45, and CD20 [Figure 2]b, [Figure 2]c, [Figure 2]d and negative for CD3, MUM1, and CK. Hence, a diagnosis of primary scalp lymphoma, histologic subtype diffuse large B cell lymphoma (DLBCL, Cell of Origin- Germinal Center B-cell) with HIV infection was made. | Figure 2: (a) Hematoxylin and eosin section from the scalp biopsy at high power magnification shows dyscohesive sheets of atypical lymphoid cells in a collagenous background. (b–d) On IHC, these atypical lymphoid cells show positivity for CD45, CD20, CD10
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The patient was counseled regarding therapeutic options. The patient was unwilling for injectable chemotherapy. Hence, he was first started on anti-retroviral treatment (ART), followed by oral metronomic chemotherapy. There was a near complete response (nCR) in the size of scalp mass following three months of therapy [Figure 1]c and [Figure 1]d. His CD4 cell count also increased to 450 cells/μL.
Lymphoma presenting as a tumor originating from the scalp is quite uncommon. In a case series of 398 patients from Taiwan, Chui et al. reported that only 4.1% of the patients with malignant scalp lesions had lymphoma. They reported that basal cell carcinoma (41.2%) and squamous cell carcinoma (16.6%) were the most common scalp malignancies followed by metastases, adnexal tumors, angiosarcoma, and lymphoma, in decreasing order of prevalence.[1]
Non-Hodgkin's lymphoma (NHL) is one of the AIDS-defining malignancies. Systemic AIDS-related NHLs are mostly nodal diseases. Extranodal involvement in the form of gastrointestinal tract (GIT), skin, liver, lung, and central nervous system (CNS) can also be seen. Skin is the second most common extranodal site after GIT. Differentials that need to be considered in an HIV-positive patient presenting with a scalp swelling are primary scalp lymphoma, cranial vault lymphoma, squamous cell carcinoma (SCC), basal cell carcinoma (BCC), and Kaposi's sarcoma involving the scalp. An indolent course, absence of extensive osteolytic lesions, or focal neurological deficits differentiate primary scalp lymphoma from primary cranial vault lymphoma.[2] Both BCC and SCC lesions have an increased tendency toward ulceration. Involvement of the scalp by Kaposi's sarcoma is rare. The subtype of a tumor can be obtained only by histopathological examination with immunohistochemistry. As the scalp is an easily accessible site, a core-needle biopsy can establish a diagnosis rapidly.
We emphasize that scalp lymphoma has an excellent prognosis compared to carcinoma or sarcoma. Timely anticipation, pathologic diagnosis, and an early initiation of lymphoma-directed therapy can lead to favorable outcomes in patients with DLBCL arising from the scalp region.
Authors contributions
AS and GP drafted the manuscript, managed the patient, and obtained radiology and clinical images. RS reviewed the histopathology and obtained pathologic images. All authors edited the manuscript and approved the final draft.
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 initials 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 | |  |
| 1. | Chiu CS, Lin CY, Kuo TT, Kuan YZ, Chen MJ, Ho HC, et al. Malignant cutaneous tumors of the scalp: A study of demographic characteristics and histologic distributions of 398 Taiwanese patients. J Am Acad Dermatol 2007;56:448-52.  |
| 2. | Martin J, Ramesh A, Kamaludeen M, Udhaya, Ganesh K, Martin JJ. Primary Non-Hodgkin's lymphoma of the scalp and cranial vault. Case Rep Neurol Med 2012;2012:616813. doi: 10.1155/2012/616813. |
Correspondence Address:
Gaurav Prakash,
4th Floor, F Block, Department of Clinical Hematology and Medical Oncology, PGIMER, Chandigarh
India
 Source of Support: None, Conflict of Interest: None DOI: 10.4103/ijpm.ijpm_778_22
[Figure 1], [Figure 2] |
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