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NEURORADIOLOGY Table of Contents   
Year : 2004  |  Volume : 14  |  Issue : 4  |  Page : 385-387
Primary cutaneous non-Hodgkin lymphoma of the scalp : A case report


Department of Radio diagnosis Lady Hardinge Medical College And assoc. Smt.S.K.Hospital, New Delhi, India

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Keywords: Non Hodgkin′s lymphoma, CT, Scalp Swelling

How to cite this article:
Madan R, Narula M K, Anand R, Gurtoo A. Primary cutaneous non-Hodgkin lymphoma of the scalp : A case report. Indian J Radiol Imaging 2004;14:385-7

How to cite this URL:
Madan R, Narula M K, Anand R, Gurtoo A. Primary cutaneous non-Hodgkin lymphoma of the scalp : A case report. Indian J Radiol Imaging [serial online] 2004 [cited 2014 Dec 25];14:385-7. Available from: http://www.ijri.org/text.asp?2004/14/4/385/28678

   Introduction Top


Primary cutaneous non-Hodgkin lymphoma presenting with isolated infiltration of the scalp is rare. We herein present the imaging findings in a case of diffuse primary cutaneous B-cell lymphoma of the scalp with soft-tissue masses and variable bone (calvarial) destruction. The characteristic neuro-radiological findings of this lesion are described and relevant differential diagnosis of malignant lymphoma of the scalp are discussed.


   Case Report Top


A 70 year-old woman presented with a large soft tissue mass on her scalp which had developed over the previous 1 year. The swelling first appeared behind the left ear slowly increasing in size from approximately 3x3cms to 7x7cms, extending from the nape of neck to the forehead and cheeks anteriorly. The patient had experienced significant weight loss recently but gave no history of fever, ear discharge, TB or syphilis. On examination the subcutaneous scalp lump was non-tender, non-fluctuant and the overlying skin appeared red. No lymph nodes could be palpated in the neck or axilla. Clinical evaluation & indirect laryngoscopy suggested palsies of cranial nerves IX, X, XII. Despite multiple cranial nerve palsies, there was no evidence of leptomeningeal disease on CSF examination and serology for HIV was negative. Peripheral blood counts & bone marrow aspiration study were normal.

Computed tomographic examination showed a diffuse hyperdense mass of the scalp in the left parieto-occipital region extending anteriorly to involve the frontal region and across the midline to completely cover the cranial vault superiorly[Figure - 1],[Figure - 2] .The underlying left occipital bone showed lytic lesions and scalloping [Figure - 3]. In addition, diffuse infiltration of the left posterior cervical space, pinna & external auditory meatus, infra-temporal fossa and the skull base was also seen . There was no concurrent nodal or intracranial (extradural/meningeal) involvement or invasion of the orbit. Further staging workup, viz: CT of the chest and abdomen/pelvis failed to reveal any other sites of disease. Histologic and immunopathologic examinations revealed diffuse primary cutaneous B-cell lymphoma of the scalp( according to the Revised European-American Lymphoma (REAL) classification) The patient was treated with chemotherapy(-CHOP cyclophosphamide, doxorubicin, vincristin predonisolone) and showed good response with regression of the cutaneous lesions.

Most primary cutaneous B-cell lymphomas(PCL) have been reported involving the head & neck region and therefore PCL of the scalp need to be differentiated from primary malignant non-Hodgkin's lymphoma of cranial vault which are more frequently associated with intra-cranial (extradural) extension[1]. Other important clinicopathologic & radiological differential diagnoses include protruding tumorous angiolymphoid hyperplasia with eosinophilia (ALHE), kimura's disease of the scalp, pseudolymphoma, cranial vault meningioma, and metastasis etc.[2],[3]

The main clinico-radiological differences between PCL of the scalp and primary NHL of the cranial vault include a shorter duration of symptomatology and early onset of focal neurological deficits , large soft-tissue mass and extensive osteolytic lesions in primary NHL of the cranial vault . As seen in our case, the disease ran an indolent course over a period of one year with no intra-cranial/orbital invasion and subtle destruction of underlying bones by the diffusely infiltrating soft tissue mass.[4] Notwithstanding - primary malignant lymphoma originating from the skull may extend outside the cranium first and within the cranium subsequently and therefore at presentation more than half of the patients report with a scalp mass rather than any neurological sign. Conversely, subcutaneous malignant lymphoma may involve the underlying skull & dura eventually [5],[6]

Most PCL's are hyperdense on unenhanced CT and show marked enhancement on postcontrast studies. Magnetic resonance imaging is important for evaluating loco-regional spread - to see involvement of bone marrow, leptomeninges or dural venous sinuses. .[7] Gallium scanning can rule out or confirm extra-cutaneous involvement and the presence or absence of activity in cases of Gallium avid lymphomas is helpful in indicating the nature of residual soft tissue masses following chemotherapy/radiotherapy.

Skin is the second most common site of extranodal lymphoma. Among them, 65% are T-cell lymphomas(Mycosis fungoides being the most common form of low-grade malignant peripheral cutaneous T-cell lymphoma ), 25% B-cell lymphomas and about 10% rare variants or non classifiable lymphomas.. Only 15 to 25% of cutaneous lymphomas show extra cutaneous manifestations at time of diagnosis. The prognosis is relatively good, since the average survival time from diagnosis is 12 to 14 years[8]

We are reporting this case because of its rare occurrence. A diagnosis of primary lymphoma of the skull vault should always be considered in the differential diagnosis when such a lesion is encountered in elderly patients.

Most primary cutaneous B-cell lymphomas(PCL) have been reported involving the head & neck region and therefore PCL of the scalp need to be differentiated from primary malignant non-Hodgkin's lymphoma of cranial vault which are more frequently associated with intra-cranial (extradural) extension[1]. Other important clinicopathologic & radiological differential diagnoses include protruding tumorous angiolymphoid hyperplasia with eosinophilia (ALHE), kimura's disease of the scalp, pseudolymphoma, cranial vault meningioma, and metastasis etc.[2],[3]

The main clinico-radiological differences between PCL of the scalp and primary NHL of the cranial vault include a shorter duration of symptomatology and early onset of focal neurological deficits , large soft-tissue mass and extensive osteolytic lesions in primary NHL of the cranial vault . As seen in our case, the disease ran an indolent course over a period of one year with no intra-cranial/orbital invasion and subtle destruction of underlying bones by the diffusely infiltrating soft tissue mass.[4] Notwithstanding - primary malignant lymphoma originating from the skull may extend outside the cranium first and within the cranium subsequently and therefore at presentation more than half of the patients report with a scalp mass rather than any neurological sign. Conversely, subcutaneous malignant lymphoma may involve the underlying skull & dura eventually [5],[6]

Most PCL's are hyperdense on unenhanced CT and show marked enhancement on postcontrast studies. Magnetic resonance imaging is important for evaluating loco-regional spread - to see involvement of bone marrow, leptomeninges or dural venous sinuses. .[7] Gallium scanning can rule out or confirm extra-cutaneous involvement and the presence or absence of activity in cases of Gallium avid lymphomas is helpful in indicating the nature of residual soft tissue masses following chemotherapy/radiotherapy.

Skin is the second most common site of extranodal lymphoma. Among them, 65% are T-cell lymphomas(Mycosis fungoides being the most common form of low-grade malignant peripheral cutaneous T-cell lymphoma ), 25% B-cell lymphomas and about 10% rare variants or non classifiable lymphomas.. Only 15 to 25% of cutaneous lymphomas show extra cutaneous manifestations at time of diagnosis. The prognosis is relatively good, since the average survival time from diagnosis is 12 to 14 years[8]

We are reporting this case because of its rare occurrence. A diagnosis of primary lymphoma of the skull vault should always be considered in the differential diagnosis when such a lesion is encountered in elderly patients.

 
   References Top

1.Franco R, Fernandez-Vazquez A, Rodriguez-Peralto JL, Bellas C, Lopez-Rios F, Saez A, Villuendas R, Navarrete M, Fernandez I, Zarco C, Piris MA Cutaneous follicular B-cell lymphoma: description of a series of 18 cases.Am J Surg Pathol. 2001 Jul;25(7):875-83.  Back to cited text no. 1    
2.Isla A, Alvarez F, Gutierrez M, Gamallo C, Garcia-Blazquez M, Vega A. Primary cranial vault lymphoma mimicking meningioma. Neuroradiology. 1996 Apr;38(3):211-3  Back to cited text no. 2    
3.Kobayashi H, Moriya T, Tagami H. Protruding tumorous angiolymphoid hyperplasia with eosinophilia (ALHE) of the scalp accompanied by arterial occlusion. J Dermatol. 2002 Jan;29(1):38-42.  Back to cited text no. 3    
4.Kantarci M, Erdem T, Alper F, Gundogdu C, Okur A, Aktas A. Imaging characteristics of diffuse primary cutaneous B-cell lymphoma of the cranial vault with orbital and brain invasion.AJNR Am J Neuroradiol. 2003 Aug;24(7):1324- 6  Back to cited text no. 4    
5.Mongia S, Shukla D, Devi BI, Reddy TV. Primary cranial vault non-Hodgkin's lymphoma.Neurol India. 2003 Jun;51(2):293-4.  Back to cited text no. 5    
6.Thurnher MM, Rieger A, Kleibl-Popov C, Schindler E. Malignant lymphoma of the cranial vault in an HIV-positive patient: imaging findings.Eur Radiol. 2001;11(8):1506- 9.  Back to cited text no. 6    
7.Duyndam DA, Biesma DH, van Heesewijk JP. Primary non-Hodgkin's lymphoma of the cranial vault; MRI features before and after treatment. Clin Radiol. 2002 Oct;57(10):948-50.  Back to cited text no. 7    
8.Burg G, Dummer R, Kerl H. Classification of cutaneous lymphomas. Dermatol Clin. 1994 Apr;12(2):213-7.  Back to cited text no. 8    

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Correspondence Address:
M K Narula
Professor & Head of Department of Radio diagnosis, LHMC & assoc. hospitals) J-13/42, Rajouri Garden New Delhi-110027
India
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[Figure - 1], [Figure - 2], [Figure - 3]



 

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