Intra-abdominal inflammatory pseudotumor-like follicular dendritic cell sarcoma associated with paraneoplastic pemphigus: A case report and review of the literature

Table 1 Summary of 32 cases of paraneoplastic pemphigus-associated follicular dendritic cell sarcoma

Ref.	Sex/age	Tumor pathology	Location	Maximum diameter (cm)	Clinical manifestations	Treatments	Follow-up
Walters et al[4]	M/48	FDCS	Anterior mediastinum	NA	Lichenoid skin lesions, BO, MG	Tumor resection, multiple immunosuppressive therapies	Progressive respiratory disease
	M/88	FDCS	Retropharynx	8	Mucosal lichenoid erosions	Tumor resection	DOD within 1 yr, status unknown
	F/59	FDCS	Axillary lymph node	NA	Lichenoid skin lesions, mucocutaneous blisters, BO	Tumor resection	DOD within 6 mo
	M/23	FDCS associated with CD	Cervical lymph node	NA	Mucosal lichenoid erosions, BO	Partial tumor resection and residual mass was radiated	Tumor recurrence and DOD within 2 yr
Lu et al[5]	F/49	FDCS	Pancreatic tail	6	Stomatitis, MG, pulmonary infection	Tumor resection, antifungal and anti-infection therapies	DOD 12 d after surgery
Jonkman et al[6]	F/35	FDCS	Intra-abdomen	NA	Stomatitis, punctate keratoses with central ulceration on the palms and soles.	Tumor resection and intensive immunosuppression	DOD with respiratory failure
Akel et al[7]	M/39	FDCS	Intra-abdomen	18	Lichenoid skin lesions, mucocutaneous blisters, febrile neutropenia	Tumor resection and high-dose steroids	DOD with severe pneumonia and acidosis
Wang et al[8]	F/56	FDCS associated with CD	Retroperitoneum	10	Stomatitis, polymorphous skin lesions, BO	Tumor resection, IVIg and steroid therapies	Alive at 4 yr follow-up
Wang et al[9]	F/27	FDCS	Retroperitoneum	8	Stomatitis, conjunctivitis, lichenoid skin lesions	Tumor resection	Tumor recurrence 5 yr after surgery
Su et al[10]	M/43	FDCS	Retroperitoneum	5	Stomatitis, lichenoid skin lesions	Tumor resection and lymphadenectomy, IVIg and steroid therapies	DOD with multiple organ failure
Chow et al[11]	M/62	FDCS	Anterior mediastinum	7.5	Stomatitis, conjunctivitis, mucocutaneous blisters	Right thoracotomy and tumor resection, adjuvant radiotherapy	DOD with respiratory failure
Garza-Chapa et al[12]	M/20	FDCS	Right-side mediastinum	7	Stomatitis, conjunctivitis, lichenoid skin lesions	Right thoracotomy and tumor resection, chemotherapy with R-CVP (rituximab, cyclophosphamide, vincristine, prednisone)	Resolution of skin lesions and no evidence of tumor recurrence at 1-yr follow-up
Streifel et al[13]	M/72	FDCS	Right-side mediastinum	NA	Stomatitis, conjunctivitis, and glans penis involvement, lichenoid skin lesions, MG	Thymectomy and partial pericardiectomy, rituximab, IVIg, steroids and mycophenolate mofetil	Improvement at 9-mo follow-up
Kim et al[14]	M/68	FDCS	Small bowel mesentery	9	Abdominal palpable mass, stomatitis, conjunctivitis, MG	Tumor resection	Metastatic tumors found in the liver 1 yr after surgery; DOD within 2 yr
Seishima et al[15]	F/64	FDCS	Retroperitoneum and small intestine	15	Stomatitis, conjunctivitis, severe skin erosions	Tumor resection and steroid therapy	Tumor recurrence; DOD with fungal infective embolisms in the lungs
Liu et al[16]	F/54	FDCS	Retroperitoneum	10.8	Stomatitis, Lichenoid skin lesions	Tumor resection, systemic corticosteroid, and cyclosporine therapies	DOD with respiratory failure
Baghmar et al[17]	M/20	FDCS	Right hemipelvis	6	Stomatitis, conjunctivitis, lichenoid skin lesions	Unresectable, chemotherapy with rituximab, cyclophosphamide, adriamycin, vincristine, and prednisolone	DOD with respiratory pseudomonas infections
Hwang et al[18]	F/46	FDCS	Liver	16	Abdominal pain, Stomatitis, lichenoid skin lesions	Tumor resection, rituximab and ciclosporin therapies	Tumor recurrence 1 yr after surgery; DOD with pneumonia
Lee et al[19]	M/67	FDCS associated with CD	Small bowel mesentery	NA	Stomatitis, conjunctivitis, MG	Tumor resection, IVIg, prednisolone, and cyclosporine therapies	Metastatic tumors found in the liver 1 yr after surgery
Zhao et al[20]	F/28	IPT-like FDCS	Intra-abdomen	9	Stomatitis, blisters and erosions of the underarm, groin and perineum, and labia majora, BO	Tumor resection	Improvement after surgery
Sugiura et al[21]	M/28	FDCS associated with CD	Left retroperitoneum	7.7	Stomatitis, polymorphic cutaneous lesions, papillomatous hyperplasia on the tongue	Tumor resection, chemotherapy of COP (cyclophosphamide, vincristine, and prednisolone)	Skin lesions healed completely except for the papillomatous hyperplasia on the tongue, and follow-up is not clear
Marzano et al[22]	F/53	FDCS	Right retroperitoneum	9	Stomatitis, conjunctivitis, lichenoid skin lesions, dyspnea	Tumor resection, systemic corticosteroid and IVIg therapies	DOD with respiratory failure within 2 yr
Meijs et al[23]	M/60	FDCS	Mediastinum	6	Stomatitis, conjunctivitis, mucocutaneous blisters and erosions, BO	Tumor resection, chemotherapy with rituximab, IVIg, plasmapheresis, corticosteroid, azathioprine and cyclophosphamide	DOD with respiratory failure
Lee et al[24]	M/66	FDCS associated with CD	Right retroperitoneum	12	Stomatitis, conjunctivitis, mucocutaneous blisters and erosions, lichenoid skin lesions	Tumor resection and antibiotics therapy	DOD with sepsis 8 d after surgery
Choi et al[25]	F/39	FDCS	NA	NA	Stomatitis, mucocutaneous blisters and erosions	Tumor resection	Alive for 5 yr, and skin lesions healed except for oral persistent mucositis
Zhang et al[26]	M/20	FDCS associated with CD	NA	NA	Stomatitis, conjunctivitis and genital involvement, skin blisters	Tumor resection	Alive for 3 yr without recurrence
	M/16	FDCS associated with CD	NA	NA	Stomatitis, conjunctivitis and genital involvement, skin blisters	Tumor resection	DOD with severe infections 2 wk after surgery
Yamada et al[27]	M/68	FDCS	Retroperitoneum	NA	Stomatitis, conjunctivitis and genital involvement, polymorphic cutaneous lesions	Tumor resection, plasmapheresis, and steroid pulse therapies	DOD with septicemia 2 mo after surgery
Ogawa et al[28]	M/28	FDCS	Retroperitoneum	NA	Stomatitis, skin blisters and erosions	Partial resection, IVIg, chemotherapy with cyclophosphamide, vincristine, and prednisolone therapies	Alive for 3 yr, and skin lesions healed except for oral persistent mucositis
Raco et al[29]	F/61	FDCS associated with CD	Intra-abdomen	10	Stomatitis, lichenoid skin lesions, dyspnea	Previous: splenectomy and chemotherapy 3 yr agoRecent: IVIg, rituximab, steroid and antibiotic therapies	Tumor metastasis or recurrence; DOD with respiratory failure
Rice et al[30]	F/41	FDCS	Retroperitoneum	8	Stomatitis, conjunctivitis, lichenoid skin lesions dyspnea,	Tumor resection, IVIg, systemic corticosteroid, rituximab, and daclizumab therapies	Progressive respiratory failure
Wang et al[31]	F/60	IPT-like FDCS	Left axillary and cervical lymph nodes	6.4	Stomatitis, polymorphic cutaneous lesions, MG, dyspnea	Tumor resection, IVIg, steroid, and rituximab therapies	DOD with multiple organ failure
Present case	F/27	IPT-like FDCS	Intra-abdomen	9	Stomatitis, conjunctivitis, skin blisters and erosions, mild dyspnea	Tumor resection, tapering corticosteroid	No evidence of tumor recurrence at 1-yr follow-up

M: Male; F: Female; DOD: Dead of disease; NA: Not available; FDCS: Follicular dendritic cell sarcoma; CD: Castleman disease; BO: Bronchiolitis obliterans; MG: Myasthenia gravis; IVIg: Intravenous immunoglobulin.