An unusual presentation of primary cutaneous follicle center lymphoma

Janowska A.*, Fidanzi C., Granieri G., Iannone M., Bonadio A.G.

Summary. Primary cutaneous follicle center lymphoma (PCFCL) is a rare low-grade cutaneous B-cell lymphoma. Clinically, PCFCL is usually an erythematous subcutaneous nodule or an infiltrated plaque. The dermoscopy is non-specific and it is characterized by polymorphous vascular pattern, arborizing vessels over a salmon-colored background and white areas. We reported a case of a 36-year-old woman presented with a rapidly growing, flashed-color, exophytic, soft consistency nodule on her scalp. Dermoscopy showed a diffuse structureless, skin-color area associated with a rare arborizing vascular pattern and brown circles. We reported a peculiar clinical and dermoscopic variant. This clinical presentation of PCFCL is unusual and represents a pitfall in the early clinical diagnosis. Histopathology is mandatory for a correct diagnosis.

Submitted: January 4, 2021.
*Correspondence: E-mail: dottoressajanowska@gmail.com
Abbreviation used: PCFCL — primary cutaneous follicle center lymphoma.

DOI: 10.32471/exp-oncology.2312-8852.vol-43-no-4.17026

Primary cutaneous follicle center lymphoma (PCFCL) is a rare low-grade cutaneous B-cell lymphoma. Clinically, PCFCL is usually an erythematous subcutaneous nodule or an infiltrated plaque that slowly expands to the surrounding area. The most frequent anatomic sites of PCFCL development are head, neck and back. The diagnosis is based on clinical, histopathological, immunohistochemical features [1]. The 5-year survival rate is > 95%, although PCFCL can rarely present with systemic involvement or evolve in diffuse large B-cell lymphoma [2].

CASE REPORT

We reported a case of a 36-year-old woman presented with a rapidly growing, flashed-color, exophytic, soft consistency, 1 cm nodule on her scalp. The lesion appeared 6 months before admission (Fig. 1). Dermoscopy showed a diffuse structureless, skin-color area associated with a rare arborizing vascular pattern and brown circles (Fig. 2).

 An unusual presentation of primary cutaneous follicle center lymphoma
Fig. 1. Clinical aspects of the lesion. The lesion appears as flashed-color, exophytic, soft consistency, nodule on the scalp
 An unusual presentation of primary cutaneous follicle center lymphoma
Fig. 2. Dermoscopic aspect of the lesion. Dermoscopy showed a diffuse structureless, skin-color area associated with a rare arborizing vascular pattern and brown circles

Histology revealed dense proliferations of small-medium lymphocytes, which are separated from epidermis by grenz zone. The lymphocytes showed a follicular diffuse growth pattern and a spread to hypodermis. After a high microscopic examination, we observed irregular and abnormal follicle, where we reported large centrocytes with cleaved/irregular nuclei and inconspicuous nucleoli and centroblasts with larger, rounder nuclei, more open chromatin, and some peripheral nucleoli. Immunohistochemistry revealed that neoplastic lymphocytes expressed CD20, CD79a, Bcl-6 and PAX5 while Bcl-2, CD10, MUM1, CD5, CD23 were negative. Ki-67 revealed a high proliferation index in central follicles and a diffuse growth pattern. The diagnosis of PCFCL was based on histopathology (Fig. 3) and immunohistochemistry (Fig. 4).

 An unusual presentation of primary cutaneous follicle center lymphoma
Fig. 3. PCFCL with a diffuse growth pattern: a — ×10 (H&E); b — ×20 (H&E) of the deeper part of the lesion. Diffuse growth pattern is evident
 An unusual presentation of primary cutaneous follicle center lymphoma
Fig. 4. PCFCL nodular pattern: a — immunostaining (CD20), ×20; b — immunostaining (CD3), × 20, deep part of the lesion; c — immunostaining (Bcl-2), ×10, deep part of the lesion; d — immunostaining (Bcl-6), ×20, deep part of the lesion (follicle-center)

A complete blood cell count, a comprehensive metabolic panel, the lactate dehydrogenase, and the positron emission tomography/computed tomography scans of the chest/abdomen/pelvis with contrast excluded visceral involvements.

DISCUSSION

PCFCL is an indolent cutaneous B-cell lymphoma with a prevalence of 10–20% of all skin lymphomas. The known risk factors include Borrelia burgdorferi, Helicobacter pylori, and Epstein — Barr virus infections [3]. PCFCL generally develops as erythematous subcutaneous nodules or infiltrated plaques. Peculiar clinical variants have been described as miliary papules [4] and scar alopecia [5]. Local recurrence is reported in 20–30% of cases [3]. Differential diagnosis includes psedolymphomas, pyogenic granuloma, spitz nevus, nodular melanoma, basal cell carcinoma, adnexal tumors, Merkel cell carcinoma and other cutaneous lymphomas [6].

The treatment consists of surgery, radiotherapy, systemic rituximab, chemotherapy [7] and intralesional interferon-α [8] and rituximab [9]. To our knowledge, dermoscopic evaluation of PCFCL has been limited. The dermoscopy is non-specific and it is characterized by polymorphous vascular pattern, arborizing vessels over a salmon-colored background and white areas [10]. We reported a peculiar clinical and dermoscopic variant. This clinical presentation of PCFCL is unusual and represents a pitfall in the early clinical diagnosis. Although the rapid growth of this lesion may be indicative of a malignant tumor, the clinical and dermoscopic aspects may mimic benign lesion. It is possible to misdiagnose PCFCL as a benign lesion when it presents itself as a solitary flashed-color, soft consistency nodule, therefore histopathology is mandatory for a correct diagnosis.

REFERENCES

1. Skala SL, Hristov B, Hristov AC. Primary cutaneous follicle center lymphoma. Arch Pathol Lab Med 2018; 142: 1313–21. doi: 10.5858/arpa.2018-021-RA
2. Goodlad JR, Krajewski AS, Batstone PJ, et al. Scotland and Newcastle Lymphoma Group. Primary cutaneous follicular lymphoma: a clinicopathologic and molecular study of 16 cases in support of a distinct entity. Am J Surg Pathol 2002; 26: 733–41. doi: 10.1097/00000478-200206000-0006
3. Chian NYZ, Uddin A, Abdullah A, et al. Primary cutaneous diffuse large B-cell lymphoma, leg type: Two Bcl-2 negative cases. J Am Acad Dermatol 2013; 68: AB48.
4. Salas-Gianini A, Ahumada FV, Vial AL, et al. Miliary and agminated papules: report of an atypical presentation of primary cutaneous follicle center lymphoma. Int J Dermatol 2020; 59: e409–11. doi: 10.1111/ijd.1198
5. Ahmed AA, Almohanna H, Griggs J, et al. Unusual clinical presentation of a primary cutaneous follicle center lymphoma on the scalp of a middle-aged female: case report and review of the literature. Skin Appendage Disord 2019; 5: 379–85. doi: 10.1159/00050174
6. Mascolo M, Piccolo V, Argenziano G, et al. Dermoscopy pattern, histopathology and immunophenotype of primary cutaneous B-cell lymphoma presenting as a solitary skin nodule. Dermatology 2016; 232: 203–7. doi: 10.1159/00044251
7. Hamilton SN, Wai ES, Tan K, et al. Treatment and outcomes in patients with primary cutaneous B-cell lymphoma: the BC Cancer Agency experience. Int J Radiat Oncol Biol Phys 2013; 87: 719–25. doi: 10.1016/j.ijrobp.2013.07019
8. Vandersee S, Terhorst D, Humme D, Beyer M. Treatment of indolent primary cutaneous B-cell lymphomas with subcutaneous interferon-alfa. J Am Acad Dermatol 2014; 70: 709–15. doi: 10.1016/j.jaad.2013.11019
9. Qiao J, Lu RN, Wang L, et al. Primary cutaneous follicle center cell lymphoma: a case report and literatures review. Zhonghua Xue Ye Xue Za Zhi 2018; 39: 328–30. doi: 10.3760/cma.j.issn.0253-2727.2018.04015
10. Geller S, Marghoob AA, Scope A, et al. Dermoscopy and the diagnosis of primary cutaneous B-cell lymphoma. J Eur Acad Dermatol Venereol 2018; 32: 53–6. doi: 10.1111/jdv.14549

НЕТИПОВИЙ ВИПАДОК ПЕРВИННОЇ ШКІРНОЇ ФОЛІКУЛЯРНОЇ ЛІМФОМИ

A. Яновська*, C. Фіданці, Г. Граньєрі, M. Яннон, A.Г. Бонадіо

Університет м. Піза, Піза 56127, Італія

Резюме. Первинна шкірна фолікулярна лімфома (ПШФЛ) є рідкісною низькозлоякісною шкірною В-клітинною лімфомою. Клінічно ПШФЛ представлена зазвичай еритематозним підшкірним вузликом або інфільтрованою бляшкою. Дермоскопічні ознаки є неспецифічними і характеризуються поліморфною васкулярною картиною з судинами, що розгалужуються на рожевому тлі з білими ділянками. Описано випадок ПШФЛ у 36-річної жінки. Новоутворення на волосистій частині голови характеризувалося швидким екзофітним ростом, було м’ясистого вигляду та м’якої консистенції. Дермоскопічно виявлено дифузну безструктурну ділянку шкірного забарвлення з розгалуженими судинами та коричневими колами. Цей клінічний випадок був нетиповим з клінічного та дермоскопічного погляду, що демонструє можливі складності для ранньої клінічної діагностики. Тому гістопатологічне дослідження є обов’язковим для правильної діагностики.

Ключові слова: первинна шкірна фолікулярна лімфома, дермоскопія (клінічні аспекти), дермопатологія (складнощі діагностики).

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