Paraneoplastic pemphigus associated with follicular dendritic cell sarcoma: report of a case and review of literature

Abstract

Follicular dendritic cell sarcoma (FDCS) is a rare tumor associated with paraneoplastic pemphigus. It is Blame drenchs auxiliary cell tumor which is derived from the peripheral lymphoid tissues. Throughout the world, several patients of paraneoplastic pemphigus associated follicular dendritic cell sarcoma were reported in the literature, but mostly originated from the neck lymph nodes, and extranodal origin of follicular dendritic sarcoma was rarely reported. Also, so far we have found that the malignant degree of all patients diagnosed with malignant tumors have been reported were low and after combined treatment of surgery, radiotherapy and chemotherapy, most of the prognosis was good. However, here we present a patient of paraneoplastic pemphigus associated with follicular dendritic cell sarcoma origined from outside of the lymph nodes and had high tumor malignant degree for its unclear cell boundaries, obvious atypia and mitoses and the patient's state became progressively deteriorate after operation.

Keywords: Paraneoplastic; follicular dendritic cell sarcoma; pemphigus.

Figure 1

Paraneoplastic pemphigus patients with head and neck, chest and abdomen, back of the hand and dorsal pedis skin lesions. A. The mouth and neck swelling, erosion, surface blood scab; B. Thoracic and abdominal large dense punctate erosions with thick scales, partial blister; C. Dense punctate erosions, papules and macula presented on the back of the hands; D. dorsal scattered sheet erosion.

Figure 2

Endoscopic findings: the basal layer blister or fracture, basal cells showed villous protrusion into epidermal direction above the lysis of the cells, scattered in the stratum spinosum, lesions involved the hair follicle epithelial; lymphocytes, histiocytes, eosinophilic infiltrate into the superficial dermal perivascular.

Figure 3

Results of MRI (A-C) and results of CT (D) (the arrows show tumor’s location).

Figure 4

A view of the position and shape of the tumor during the operation and surgical removal of the lymph nodes (A-D) (B arrow shows the location of tumor, D arrow shows the removed lymph nodes).

Figure 5

Paraneoplastic pemphigus patients with head and neck, chest and abdomen, back of the hand and dorsal pedis skin lesions decreased obviously after operation. A. The mouth and neck swelling, erosion and surface blood scab was less than before; B. Thoracic and abdominal large dense punctate erosions with thick scales, partial blister; C. Dense punctate erosions, papules and macula presented on the back of the hands; D. Dorsal scattered erosion.

Figure 6

About a week after surgery, the patient’s state became progressively deteriorate with large area of skin erosion, bleeding, accompanied by an unpleasant odor, the incision burst open with peritoneum and intestine protruding (the arrows show protruding intestine). A. six days after operation; B. seven days after operation.

Figure 7

The tumor was composed of proliferation of spindle cells and oval cells, with unclear cell boundaries, obvious atypia and mitoses.

Figure 8

Resutl of HE. A. cells were wispy, swirling 360 degrees and multinucleated cells were visible. B. Remnants of small lymphocytes distributed around the perivascular.

Figure 9

Immunohistochemical follicular dendritic cell markers of the patient’ specimens: A: CD21 (+); B: CD23 (+); C: Vimentin (+); D: EMA (+); E: Ki-67 about 3% (+).