Features of oral peripheral T-cell lymphoma, not otherwise specified

doi:10.3969/j.issn.1674-8115.2025.05.015

Abstract

Abstract:

Objective ·To investigate the clinical manifestations and immunophenotypic features of peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS), involving the oral cavity. Methods ·The medical histories and pathology records of patients diagnosed with oral PTCL-NOS in the Department of Oral Mucosal Diseases of Shanghai Ninth People′s Hospital, Shanghai Jiao Tong University School of Medicine, between August 2020 and August 2024 were retrospectively analyzed. In addition, 5 databases, including PubMed, Web of Science, Embase, Scopus, and CNKI, were searched, and relevant cases reported internationally from January 2014 to September 2024 were reviewed. Results ·A total of 20 oral PTCL-NOS cases were included, comprising 11 males (55.0%) and 9 females (45.0%). The patients′ ages at initial diagnosis ranged from 25 to 77 years, with a mean age of (52.53±12.94) years. The most common sites were the tongue (25.0%), palate (25.0%), and buccal mucosa (20.0%). Nineteen cases (95.0%) had no B symptoms. The cases were positive for CD3 (19/19), CD4 (11/13), CD8 (7/12), CD2 (5/6), CD7 (5/5), TIA-1 (6/7), GB (9/13), perforin (4/6). EBER expression was negative (8/8). The Ki-67 proliferation index was ≥60% in 85% of cases. Conclusions ·Oral PTCL-NOS is extremely rare and has an aggressive clinical behavior. The oral manifestation presents as deep and large mucosal ulcers with uneven bases, and nodules can be palpable. The pathological features are heterogeneous. Immunophenotype detection is useful for early diagnosis and classification. It is essential for stomatologists to enhance their awareness of this malignancy to avoid delayed diagnosis and treatment.

Key words: oral feature, peripheral T-cell lymphoma (PTCL), not otherwise specified (NOS), clinical pathology

CLC Number:

R733.4

HUANG Runyu, ZHANG Chunye, ZHANG Ying, ZHAO Zhengyan, YANG Yang, WU Lan. Features of oral peripheral T-cell lymphoma, not otherwise specified[J]. Journal of Shanghai Jiao Tong University (Medical Science), 2025, 45(5): 653-660.

Figures/Tables 6

TrendMD

References 22

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Year	Patient/ reference	Gender/ age	Location	B symptom	Radiographic feature	Ann Arbor staging	Treatment
2023	Case 1	F/55	Tongue	None	MRI showed a vaguely-bordered soft tissue thickening at the anterior tongue. In addition, MRI showed an intermediate T1 signal and slightly high T2 signal lesion at the right side of the tongue, which was about 3.6 cm×1.3 cm. The lesion demonstrated heterogeneous contrast enhancement	Ⅰ EA	/
2024	Case 2	M/44	Hard palate	None	MRI illustrated a vaguely-bordered soft tissue thickening, which measured approximately 3.4 cm×2.7 cm×1.2 cm and showed a slightly low T1 signal and slightly high T2 signal. The lesion demonstrated heterogeneous contrast enhancement. There were several enlarged lymph nodes in the level Ⅱ‒level Ⅳ cervical	Ⅳ A	GemOx-D
2014	1/[2]	M/59	The right side of the tongue base	None	CT scan presented an ill-defined, heterogeneous enhancing soft tissue mass lesion in the right tongue base and enlarged lymph nodes in the right level Ⅱ	Ⅱ A	CHOP+RT+VMAT
2016	2/[3]	F/50	The left side of the anterior tongue	None	MRI T2WI showed a bordered lesion at the anterior tongue	Ⅰ	None
2017	3/[4]	F/42	Near the median sulcus of the tongue	None	Enhanced MRI showed a bordered, bar-shaped, irregular lesion with intermediate T1 signal and slightly high T2 signal in the right portion of the tongue, and its size was about 8.5 mm×24.5 mm	/	Surgery, CHOP
2018	4/[5]	F/25	Upper lip, palate, and maxillary sinus	Fever, weight loss	CT scan with contrast showed an expansive and infiltrative formation with irregular contours and ulceration, extending from the upper lip to the nasolabial sulcus, with infiltration into the epidermis and nasal mucosa. The formation affected the right tear duct without bone involvement. In addition, CT showed a bilateral increase in the number and size of the cervical lymph nodes, mainly the submandibular chain	Ⅳ B	CHOEP
2020	5/[6]	M/ 75‒80	The right side of the tongue base	None	CT scan showed a well-bordered cystic mass (2 cm in diameter) at the right base of the tongue extending into the pharynx	Ⅳ A	GDP+CHOP
2021	6/[7]	F/63	Submandibular region	/	/	/	/
2021	7/[7]	M/67	Buccal mucosa	/	/	/	/
2021	8/[7]	M/57	Hard palate	/	/	/	/
2021	9/[7]	M/38	Soft palate	/	/	/	/
2021	10/[7]	M/59	Buccal mucosa	/	/	/	/
2021	11/[7]	F/56	Tonsil	/	/	/	/
2021	12/[7]	F/66	Tonsil	/	/	/	/
2023	13/[8]	M/34	Right buccal mucosa	None	CT scan imaging showed destructive and expansive mass with central necrosis, extending to maxillary and ethmoid sinuses	Ⅱ EA	CHOEP+RT
2024	14/[9]	F/37	Maxillary gingiva	None	/	/	Anthracycline-based regimens
2024	15/[9]	M/44	Buccal mucosa	None	/	/	/
2024	16/[9]	F/62	Palate and gingiva	None	/	/	Anthracycline-based regimens
2024	17/[9]	M/55	Palate and maxillary sinus	None	CT scan illustrated the neoplasm invading the maxillary sinus and the orbital cavity, and extending toward the base of the skull	/	Anthracycline-based regimens
2024	18/[9]	M/61	Soft palate and tonsil	None	/	/	/

Year	Patient/ reference	Gender/ age	Location	B symptom	Radiographic feature	Ann Arbor staging	Treatment
2023	Case 1	F/55	Tongue	None	MRI showed a vaguely-bordered soft tissue thickening at the anterior tongue. In addition, MRI showed an intermediate T1 signal and slightly high T2 signal lesion at the right side of the tongue, which was about 3.6 cm×1.3 cm. The lesion demonstrated heterogeneous contrast enhancement	Ⅰ EA	/
2024	Case 2	M/44	Hard palate	None	MRI illustrated a vaguely-bordered soft tissue thickening, which measured approximately 3.4 cm×2.7 cm×1.2 cm and showed a slightly low T1 signal and slightly high T2 signal. The lesion demonstrated heterogeneous contrast enhancement. There were several enlarged lymph nodes in the level Ⅱ‒level Ⅳ cervical	Ⅳ A	GemOx-D
2014	1/[2]	M/59	The right side of the tongue base	None	CT scan presented an ill-defined, heterogeneous enhancing soft tissue mass lesion in the right tongue base and enlarged lymph nodes in the right level Ⅱ	Ⅱ A	CHOP+RT+VMAT
2016	2/[3]	F/50	The left side of the anterior tongue	None	MRI T2WI showed a bordered lesion at the anterior tongue	Ⅰ	None
2017	3/[4]	F/42	Near the median sulcus of the tongue	None	Enhanced MRI showed a bordered, bar-shaped, irregular lesion with intermediate T1 signal and slightly high T2 signal in the right portion of the tongue, and its size was about 8.5 mm×24.5 mm	/	Surgery, CHOP
2018	4/[5]	F/25	Upper lip, palate, and maxillary sinus	Fever, weight loss	CT scan with contrast showed an expansive and infiltrative formation with irregular contours and ulceration, extending from the upper lip to the nasolabial sulcus, with infiltration into the epidermis and nasal mucosa. The formation affected the right tear duct without bone involvement. In addition, CT showed a bilateral increase in the number and size of the cervical lymph nodes, mainly the submandibular chain	Ⅳ B	CHOEP
2020	5/[6]	M/ 75‒80	The right side of the tongue base	None	CT scan showed a well-bordered cystic mass (2 cm in diameter) at the right base of the tongue extending into the pharynx	Ⅳ A	GDP+CHOP
2021	6/[7]	F/63	Submandibular region	/	/	/	/
2021	7/[7]	M/67	Buccal mucosa	/	/	/	/
2021	8/[7]	M/57	Hard palate	/	/	/	/
2021	9/[7]	M/38	Soft palate	/	/	/	/
2021	10/[7]	M/59	Buccal mucosa	/	/	/	/
2021	11/[7]	F/56	Tonsil	/	/	/	/
2021	12/[7]	F/66	Tonsil	/	/	/	/
2023	13/[8]	M/34	Right buccal mucosa	None	CT scan imaging showed destructive and expansive mass with central necrosis, extending to maxillary and ethmoid sinuses	Ⅱ EA	CHOEP+RT
2024	14/[9]	F/37	Maxillary gingiva	None	/	/	Anthracycline-based regimens
2024	15/[9]	M/44	Buccal mucosa	None	/	/	/
2024	16/[9]	F/62	Palate and gingiva	None	/	/	Anthracycline-based regimens
2024	17/[9]	M/55	Palate and maxillary sinus	None	CT scan illustrated the neoplasm invading the maxillary sinus and the orbital cavity, and extending toward the base of the skull	/	Anthracycline-based regimens
2024	18/[9]	M/61	Soft palate and tonsil	None	/	/	/

Item	Oral squamous cell carcinoma	Major recurrent aphthous ulcer	Traumatic ulceration	Tuberculous ulcer	PTCL-NOS	ENKTL-NT
Population	Middle-aged and elderly people	Adult	Teenagers and elderly people	/	Middle-aged people	Middle-aged people
Oral clinical manifestations	Persistent ulceration with raised margins and necrotic base; indurated on palpation	Oval or round ulcers with a white or yellow pseudomembrane and a surrounding erythematous halo; 1 cm or more in diameter	Frank ulceration is surrounded by a white hyperkeratotic lesion. The location and the shape of the ulcer correspond to the stimulating factor	Deep ulcer with undermined edges and red granular base covered by septic secretion	Ulceration, mass, or necrosis	Necrotic ulceration
Location of lesions	The margin of the tongue, and the belly of the tongue	The non-keratinized and keratinized oral mucosa	In a trauma-prone site	Labial mucosa, vestibule, and tongue	Tongue, palate, and buccal mucosa	Median mucosa of the palate
Systemic signs	Lymph node swelling, pain, or weight loss	/	/	Cough, anorexia, fatigue, low-grade fever and weight loss	Lymph node swelling, extra-nodal involvement of the gastrointestinal tract and skin, anemia, eosinophilia, or hypergammaglobulinaemia	Nasal mucosal symptoms or B symptoms
Clinical course	Persistent	Self-limiting, recurrent, and periodic	Self-limiting	Infection	Aggressive	Aggressive
Complementary examinations	Biopsy	/	/	Chest X-ray, tuberculin skin test, bacterial culturing	Biopsy, blood test, bone marrow biopsy	Biopsy, blood test, bone marrow biopsy, EBER-ISH

Item	Oral squamous cell carcinoma	Major recurrent aphthous ulcer	Traumatic ulceration	Tuberculous ulcer	PTCL-NOS	ENKTL-NT
Population	Middle-aged and elderly people	Adult	Teenagers and elderly people	/	Middle-aged people	Middle-aged people
Oral clinical manifestations	Persistent ulceration with raised margins and necrotic base; indurated on palpation	Oval or round ulcers with a white or yellow pseudomembrane and a surrounding erythematous halo; 1 cm or more in diameter	Frank ulceration is surrounded by a white hyperkeratotic lesion. The location and the shape of the ulcer correspond to the stimulating factor	Deep ulcer with undermined edges and red granular base covered by septic secretion	Ulceration, mass, or necrosis	Necrotic ulceration
Location of lesions	The margin of the tongue, and the belly of the tongue	The non-keratinized and keratinized oral mucosa	In a trauma-prone site	Labial mucosa, vestibule, and tongue	Tongue, palate, and buccal mucosa	Median mucosa of the palate
Systemic signs	Lymph node swelling, pain, or weight loss	/	/	Cough, anorexia, fatigue, low-grade fever and weight loss	Lymph node swelling, extra-nodal involvement of the gastrointestinal tract and skin, anemia, eosinophilia, or hypergammaglobulinaemia	Nasal mucosal symptoms or B symptoms
Clinical course	Persistent	Self-limiting, recurrent, and periodic	Self-limiting	Infection	Aggressive	Aggressive
Complementary examinations	Biopsy	/	/	Chest X-ray, tuberculin skin test, bacterial culturing	Biopsy, blood test, bone marrow biopsy	Biopsy, blood test, bone marrow biopsy, EBER-ISH

Year	Patient/ reference	EBER	CD3	CD4	CD8	CD2	CD7	CD20	Ki-67	Others
2023	Case 1	‒	+	+	+	+	+	‒	60%‒70%	TIA-1 (partial+), GB (partial+), perforin (few cells+)
2024	Case 2	/	+	/	/	+	+	‒	>90%	TIA-1(+), GB(+), perforin(+)
2014	1/[2]	‒	+	+	+	/	/	‒	80%	TIA-1(+), GB(+)
2016	2/[3]	/	+	+	+	Few cells+	Few cells+	‒	30%‒40%	/
2017	3/[4]	‒	+	‒	+	/	/	‒	30%	TIA-1(+)
2018	4/[5]	‒	+	+	‒	+	/	‒	60%	/
2020	5/[6]	‒	+	+	‒	/	/	‒	60%	TIA-1(‒), GB(‒)
2020	6/[7]	‒	+	+	‒	/	/	‒	60%‒95%	LCA(+)
2020	7/[7]	‒	+	/	/	/	/	‒	20%	LCA(+), GB(‒), CD30(‒)
2020	8/[7]	‒	+	+	/	/	/	‒	60%‒95%
2020	9/[7]	/	+	/	/	/	/	‒	60%‒95%	GB(+)
2020	10/[7]	/	/	/	/	/	/	/	60%‒95%	CD30(+)
2020	11/[7]	/	+	+	+	‒	/	‒	60%‒95%	TIA-1(+), GB(+), perforin(+)
2020	12/[7]	/	+	+	+	+	+	‒	60%‒95%	TIA-1(+), GB(+), perforin(+), CD30(‒), PD-1(+)
2023	13/[8]	/	+	/	/	/	+	‒	70%‒80%	CD30(‒)
2024	14/[9]	/	+	/	/	/	/	/	70%	Perforin(‒), GB(‒)
2024	15/[9]	/	+	/	/	/	/	/	80%	GB(‒)
2024	16/[9]	/	+	‒	+	/	/	/	95%	Perforin(‒), GB(+), CD30(‒)
2024	17/[9]	/	+	+	‒	/	/	/	95%	GB(+), CD30(‒)
2024	18/[9]	/	+	+	‒	/	/	/	90%	GB(+), CD30(+)