J Pathol Transl Med.  2020 May;54(3):253-257. 10.4132/jptm.2019.12.17.

Morphologic variant of follicular lymphoma reminiscent of hyaline-vascular Castleman disease

• Koh J
• Jeon YK

• KMID: 2501661
• DOI: http://doi.org/10.4132/jptm.2019.12.17

Abstract

Follicular lymphoma (FL) with hyaline-vascular Castleman disease (FL-HVCD)-like features is a rare morphologic variant, with fewer than 20 cases in the literature. Herein, we report a case of FL-HVCD in a 37-year-old female who presented with isolated neck lymph node enlargement. The excised lymph node showed features reminiscent of HVCD, including regressed germinal centers (GCs) surrounded by onion skin-like mantle zones, lollipop lesions composed of hyalinized blood vessels penetrating into regressed GCs, and hyalinized interfollicular stroma. In addition, focal areas of abnormally conglomerated GCs composed of homogeneous, small centrocytes with strong BCL2, CD10, and BCL6 expression were observed, indicating partial involvement of the FL. Several other lymphoid follicles showed features of in situ follicular neoplasia. Based on the observations, a diagnosis of FL-HVCD was made. Although FLHVCD is very rare, the possibility of this variant should be considered in cases resembling CD. Identification of abnormal, neoplastic follicles and ancillary immunostaining are helpful for proper diagnosis.

Keyword

Malignant lymphoma; Follicular lymphoma; Hyaline-vascular Castleman disease; In situ follicular neoplasia

Figure

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  Fig. 1. Imaging work-up of the patient at presentation. (A) Neck ultrasonography shows a hypoechoic mass (arrow). (B) Neck computed tomography shows a well-enhanced ovoid mass at level II area (arrow). (C) 18F-positron emission tomography scan shows hypermetabolic lesion in the right neck (arrow) and no other hypermetabolic lesions.

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  Fig. 2. Excised lymph node reminiscent of hyaline-vascular Castleman disease (HVCD). Vaguely nodular pattern is noted on low power (A), where regressed lymphoid follicles (B) with expanded onion skin-like mantle zones (C) are observed. (D) Hyalinized blood vessels within follicles are frequently noted. (E) Immunostaining shows the presence of BCL2-positive, BCL6-positive, and CD10-positive cells within the regressed follicles.

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  Fig. 3. Features of follicular lymphoma. Upon careful examination, abnormally conglomerated follicles are identified (A, B), and presence of BCL2-positive, BCL6-positive, and CD10-positive neoplastic B-cells is noted (B), confirming follicular lymphoma. (C) In addition, cells having an aberrant BCL2-positive, BCL6-positive, and CD10-positive immunophenotype within otherwise normal-looking follicles are observed, indicating involvement of in situ follicular neoplasia (ISFN).

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