1.Extranodal nasal type natural killer/T-cell lymphoma of the digestive system: a clinicopathological study of thirteen cases
Panhong FAN ; Zhiquan GONG ; Linjiao JIA ; Bo YANG ; Lifu WANG ; Lingfei KONG
Chinese Journal of Pathology 2021;50(10):1122-1127
Objective:To investigate the clinicopathological features, and diagnostic and differential diagnostic characteristics of extranodal nasal type natural killer/T-cell lymphoma (ENKTCL) of the digestive system.Methods:Thirteen cases of ENKTCL in the digestive system were collected at the Henan Provincial People′s Hospital, Zhengzhou, China, from August 2000 to August 2020. The histopathological, immunohistochemical and in situ hybridization features were analyzed, as well as those of T-cell receptor (TCR) gene rearrangement in some cases. The patients were followed up.Results:There were 11 males and 2 females. The age ranged from 28 to 80 years (median=53 years). Seven cases were present in the colorectum, and 3 cases were present in the small intestine. The other three cases were in stomach, gallbladder and liver (one case each). The main clinical symptoms were fever, and abdominal pain, often accompanied by fatigue, diarrhea, hematochezia, elevated serum albumin, elevated lactate dehydrogenase, and increased peripheral blood EB virus DNA copy. Histologically, the tumor accompanied by a heavy admixture of inflammatory cells (small lymphocytes, plasma cells and histiocytes). There was diffuse dense tumor cell infiltrate, with prominent coagulative necrosis. The lymphomatous infiltrate had angiocentric and angio-necrotic changes. Immunohistochemically, lymphoid cells expressed CD3 in all cases. Some of them showed weakened/absent other T cell markers, while all of them expressed CD56 except 1 case. A few of the cases showed CD4 -/CD8 + killer T cell phenotypes. In situ hybridization showed EB virus encoded RNA (EBER) was positive in all cases. Clonal TCR gene rearrangement was not detected in all 7 cases tested. The median survival time was 9 months. Conclusions:ENKTCL of the digestive system is extremely rare. It often predisposes the patients to acute abdomen such as perforation of the gastrointestinal tract. The treatment outcomes are dismal, and the prognosis is poor. Clinical and imaging studies are often non-specific. It is also easy to be misdiagnosed as non-specific ulcers. Combined with immunohistochemistry, in situ hybridization and TCR gene rearrangement analysis and better understanding of this tumor′s clinicopathological characteristics can help improve its diagnosis and early treatment.
2.Clinicopathological features of verrucous hemangioma
Panhong FAN ; Dong LIANG ; Linjiao JIA ; Yubin GONG ; Bin SUN ; Lili FU ; Qiuyu LIU
Chinese Journal of Pathology 2021;50(12):1341-1345
Objective:To investigate the clinicopathological features, and differential diagnosis of verrucous hemangioma (VH).Methods:Twenty-eight VH cases diagnosed from 2005 to 2020 in Henan Provincial People′s Hospital, Zhengzhou, China were analyzed retrospectively. Immunohistochemical studies were used to detect diagnostic markers. The mutation status of PIK3CA (exons 9 and 20) was detected using fluorescence PCR.Results:There were 13 males and 15 females in 28 cases, with the male to female ratio of 1.0∶1.2. There were 25 patients under the age of 18 years. The age range was from 10 months to 56 years (mean, 9.7 years; median, 4.5 years). There were 17 cases occurred in the lower extremities, 7 in the upper extremities and 4 in the trunk. All 28 cases were irregular red patches on the skin, which grew slowly. Some of them were thickened with uneven surface, which was light pink or red-white. Skin lesions of the 7 cases ranged from dark red and reddish brown, with a rough and hard surface. Satellite foci were present. Microscopically, 28 cases had a wide range of pathological features. Dilated, malformed vessels were observed from dermal papilla to deep soft tissue. Among them, the dermal papillary layer was mainly composed of many proliferating and expanding thin-walled capillaries and cavernous blood vessels. Thin-walled small vessels were found in the dermal reticular layer and subcutaneous fascia layer, with no obvious endothelial cell proliferation, occasional papillary hyperplasia, and lobular distribution of the malformed vessels in the fascia layer mixed with the fibroadipose tissue. There was epidermal papillary hyperplasia with hyperkeratosis and parakeratosis, lengthening and mutual fusion of epithelial horns. Immunohistochemistry showed that CD31, CD34, ERG and WT-1 were diffusely and strongly positive. The expression of GLUT-1 was present in superficial dermal vascular endothelial cells, but undetectable in the deep layer. The PIK3CA tests of 13 cases showed that no somatic mutations were found in exons 9 and 20. Twenty-five patients were followed up for 5 months to 10 years. Seven patients underwent multiple surgical resections and plastic surgeries due to the large size, and 8 patients had recurrence.Conclusions:VH is a rare congenital vascular malformation and more commonly occurs in infants and children. It tends to appear in limbs, especially lower limbs and distal limbs. Its morphology and immunophenotype are characteristic and should be distinguished from other vascular malformations and the resolution phase of infant hemangiomas. In about one third of the cases, postoperative recurrence may occur and long-term follow-up is often required.