American Journal of Dermatopathology:
Letters to the Editor
Primary Cutaneous Spindle-Cell B-Cell Lymphoma of Follicle Center Cell Origin
Jghaimi, Faissal*; Hocar, Ouafa†; Akhdari, Nadia†; Amal, Said†; Belaabidia, Badia*
Departments of *Pathology, and
†Dermatology, Mohammed VI University Hospital Center, Cadi Ayyad University, Marrakech, Morocco
The authors have no funding or conflicts of interest to disclose.
To the Editors:
Spindle-cell B-cell lymphoma is a rare morphological variant of B-cell lymphoma that is generally associated with follicle center cell origin.1–11 It was not illustrated in the recent World Health Organization and European Organization for Research and Treatment of Cancer classification.12 The possibility of a worse prognosis of this special subtype of primary cutaneous follicle center lymphoma has been discussed1,13 but has not been confirmed yet. Herein, we describe a primary cutaneous spindle-cell B-cell lymphoma of the scalp.
An 80-year-old man had a painless, slowly growing, cutaneous lesion of the scalp with a duration of 8 months. Physical examination revealed that the neoplasm measured 9 × 8 cm showed an ulcerated masse with a poorly defined boundary (Fig. 1). No lymphadenopathy was detected during the physical examination. Results from chest x-ray and ultrasonic examination were normal. Routine blood and urine tests and liver and renal function examination did not uncover any abnormality. The patient displayed no other symptoms, and her family had no history of similar diseases. A biopsy from the giant neoplasm was performed. Histopathologic examination revealed a dense, diffuse dermal infiltrate, which involved the subcutis but clearly was separated from the epidermis by a distinct Grenz zone (Fig. 2); it was mainly composed by spindle cells that exhibited condensed chromatin and abnormal mitosis with “boomerang-like” or “spermatozoa-like” appearance (Figs. 3, 4); a minor cell population showing a centroblast-like or a large centrocyte-like morphology was also seen. Immunohistochemically, both spindle cells and centroblast/centrocyte-like cells were stained positive for CD45, CD20 (Fig. 5), CD79, and Bcl-6. On the other hand, CD3, CD10, Bcl-2, MUM1, S100 protein, HMB45, cytokeratin, and muscle-specific actin were all stained negative. Staging procedures, including a bone marrow biopsy, were negative. The tumor was completely excised after the diagnosis of lymphoma, and the patient was still alive after 4 years of follow-up. No recurrence was noticed.
In 2000, Cerroni et al1 first reported primary cutaneous spindle-cell B-cell lymphoma as a rare variant of primary cutaneous B-cell lymphoma characterized by admixed centrocytes and centroblasts with spindle-shaped, elongated nuclei and in some foci a boomerang-like or spermatozoa-like morphology. Subsequently, studies reappraised this entity as a special subtype of primary cutaneous follicle center lymphoma.3,5,6 Ries et al14 reported the only Bcl-6–negative spindle-cell B-cell lymphoma. However, that particular case was not fulfilling the criteria of Cerroni et al.1
The skin is most frequently affected, with 18 cases reported.1–6,8,11,13,14 The liver, vagina, and uterus were also affected in isolated cases.7,9,10 Primary cutaneous spindle-cell B-cell lymphoma shares several clinicopathologic features with primary cutaneous follicle center cell lymphoma. It typically affects elderly people with no apparent sex difference. It usually presents as single or occasionally multiple lesions on the head or back, for which the diameter ranges from 1 to 25 cm.
Histopathologically,1–11 spindle-cell B-cell lymphoma infiltrates the entire dermis and the subcutaneous fat but clearly spares the epidermis; it was mainly composed by neoplastic cells with prominent spindle-cell differentiation and boomerang-like or spermatozoa-like morphology and cell population showing a centroblast like or a large centrocyte like. Immunophenotyping is very important for the differential diagnosis. Spindle cells are Bcl-2−, Bcl-6+, and CD10−, which are important for differentiating them from secondary cutaneous follicular lymphoma (Bcl-2+/Bcl-6+/CD10+).8 Our case was stained positive for Bcl-6 and negative for Bcl-2 and MUM1, reminiscent of the classical description of the spindle-cell B-cell lymphoma.
Cutaneous spindle-cell B-cell lymphoma needs to be distinguished from other cutaneous malignant spindle-cell tumors, including spindle-cell carcinoma, spindle-cell melanoma, atypical fibroxanthoma, soft tissue sarcoma, and sarcomatoid leukemia.1,15
Spindle-cell shape variant of primary cutaneous follicle center cell lymphoma is extremely rare, and the data on the prognosis are sparse. The majority of reported cases lack sufficient follow-up studies, but most cases seemed to show a rather indolent behavior. Only 3 cases reported by Cerroni et al1 and Rozati et al13 showed aggressive prognosis. Our case exhibited a clinically indolent behavior as no other organ was involved during 4 years of follow-up.
In summary, by reason of there being only a few reported cases of the spindle-cell variant and within those only a few having documented course of the disease, there is the need to further investigate the possible more aggressive nature of this variant.
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13. Rozati S, Kerl K, Kempf W, et al. Spindle-cell variant of primary cutaneous follicle center lymphoma spreading to the hepatobiliary tree, mimicking Klatskin tumor. J Cutan Pathol. 2013; 40:56–60.
14. Ries S, Barr R, LeBoit P, et al. Cutaneous sarcomatoid B-cell lymphoma. Am J Dermatopathol. 2007; 29:96–98.
15. Fernandez-Flores A. Diagnosis of cutaneous sarcomatoid B-cell lymphoma: some words of caution. Am J Dermatopathol. 2009; 31:510–512.
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