Takayuki Nojima

Primary enteric-type tubulovillous adenocarcinoma arising in the renal pelvis: Letter to the Editor

To the Editor: In 1960, Hasebe et al. first demonstrated the clinicopathological features of an extremely rare case of primary mucinous adenocarcinoma arising in the renal pelvis. Following that, a substantial number of interesting case reports describing the pathological features of primary pelvic adenocarcinoma were published; however, within our thorough investigation, we found less than 100 cases reported in the English literature, with most cases reported from Asian countries. Indeed, it is well-known that transitional cell (urothelial) carcinoma accounts for up to 90% of epithelial-origin malignancies arising from the renal pelvis, whereas adenocarcinoma accounts for less than 1%. Pure adenocarcinomas of the renal pelvis are histopathologically classified as tubulovillous (71.5%), mucinous (21.5%), or papillary non-intestinal (7.0%). We herein report an extremely rare case of primary enteric-type tubulovillous adenocarcinoma arising in the markedly dilated left renal pelvis, presenting as hemorrhagic, necrotic and mucinous neoplasms, in a background of severe hydrocalycosis and hydroureter. The patient, who was a man in his early seventies with an unremarkable previous medical history, presented with gross hematuria, lumbago and back pain. CT revealed severe hydronephrosis in the left kidney, associated with two renal pelvic calculi and occupying heterogeneouslyenhanced multiple pelvic nodular lesions. However, there was no definite evidence of other tumor lesions of the neck, chest, or abdomen, including metastatic foci in the lymph nodes or other organs. The laboratory data, including the blood cell count, chemistry and tumor marker levels, were within the normal limits, with the exception of mildly elevated CRP (0.43mg/dL) and uric acid (8.1mg/dL) levels. A urinalysis revealed a low white blood cell count (1 to 4 cells per high-power field). Based on the clinical findings, the initial diagnosis by the urologists was renal pelvic urothelial carcinoma with hydronephrosis and pelvic calculi; but, the urine cytology did not suggest malignancy. However, since the possibility of malignancy could not be excluded, left nephrouretectomy was performed. On gross examination, the left kidney, measured 20 13 cm and weighed 877g. Its cut surface (Fig. 1a) showed an overtly cystically-dilated renal pelvis, filled with pelvic calculi, and papillary-projected huge mass lesions, measuring more than 5 to 7 cm in diameter, which appeared gray-whitish in color, accompanied by frequent hemorrhage, necrosis and mucin production. This hydrocalycotic and hydroureteric kidney showed a markedly thinned pre-existing renal parenchyma (Fig. 1a). Our thorough examination revealed no anomalous findings (i.e., horseshoe kidney). A microscopic examination of these tumors demonstrated a proliferation of atypical tall columnar epithelial cells, arranged predominantly in a papillary or villoglandular or fused tubular growth pattern with mucin production and focal stromal invasion (Fig. 1b). On a high-power view, these atypical cells showed enlarged hyperchromatic nuclei, prominent nucleoli and abundant eosinophilic to clear cytoplasm with characteristic brush borders (Fig. 1d). Intriguingly, in the adjacent noncancerous renal pelvic mucosa, we occasionally recognized glandular metaplasia with mild cellular atypia in the covering urothelial epithelium (Fig. 1d). In contrast, our thorough investigation revealed no apparent adenomatous components. The markedly atrophic pre-existing kidney contained a very small number of glomeruli and uriniferous tubules embedded in prominent fibrosis. Very few inflammatory cells were observed. Immunohistochemistry revealed that the abovementioned enteric-type adenocarcinoma cells were specifically positive for CK7, CK20, CDX2, b-catenin and MUC2, and focally positive for MUC5AC. As to the bcatenin, the immunoreactivity uniquely showed a membranous—but not nuclear—staining pattern (Fig. 1e). Based on all of these features, the final diagnosis was primary enteric-type tubulovillous adenocarcinoma arising in the left renal pelvis. We hypothesized that this was possibly induced by a long-term inflammatory reaction caused by the patients pelvic calculi and/or hydrocalycosis/hydroureter. To date, the patient has been followed for approximately 2 years since surgery, and remains well without any sign of recurrence. Figure 1F shows a schematic illustration of the primary pelvic adenocarcinoma observed in the present case, which involved and extended to the hydrocalycotic and hydroureteric kidney with pelvic calculi. Aggressive surgical treatment in the early stage of primary renal pelvic adenocarcinoma might be associated with a better prognosis, as this disease is associated with higher-grade (i.e., invasive characteristics) malignant tumors, with a reported overall survival rate of <50% within 2 years after surgery. It is therefore critical to establish an accurate pre-operative diagnosis based on urine cytology, the clinical utility of which has been generalized in the diagnosis of urinary tract tumors. However, there are few reports of the cytological findings of primary renal pelvic

Sinonasal NUT carcinoma: clinicopathological and cytogenetic analysis with autopsy findings

Nuclear protein in testis (NUT) carcinoma is a rare malignant neoplasm with an undifferentiated morphology. Its diagnosis is often difficult, especially as the sinonasal tract gives rise to many tumors with undifferentiated morphologies. Not many cases of sinonasal NUT carcinomas have been reported, and its clinicopathological features have not been sufficiently clarified. In this study, we performed a clinicopathological study of 4 patients with sinonasal NUT carcinoma, including wide-ranging immunohistochemical tests and cytogenetic analyses using fluorescence in situ hybridization and DNA sequencing. Autopsy findings were obtained from 2 patients. Patients ages ranged from 9 months to 66 years (median, 37 years). Three cases involved the nasal cavity; of these, 2 also involved the ethmoid sinus. One case only involved the frontal sinus. Histologically, all cases revealed undifferentiated small round cell morphology and necrosis with indistinct cell borders, vesicular chromatin, and distinct nucleoli. All patients received chemoradiotherapy; 3 died of disease 10 to 15 months after their diagnoses, while one was lost to follow-up. The 2 autopsied patients showed multiorgan metastases; interestingly, one showed cartilaginous differentiation in a metastatic lesion. Immunohistochemically, all cases were diffusely positive for NUT, p63, and Myc, and were focal for p40. The cells variably expressed epithelial markers, and CD34 was positive in one patient. Cytogenetically, all showed BRD4-NUT fusion genes, but one had a different breakpoint in each exon. Finally, a literature review indicated that sinonasal NUT carcinoma tends to involve frontal and ethmoidal sinuses more frequently than other sinonasal cancers.

An autopsy case of peripheral T cell lymphoma occurring in a postpartum woman: a unique case suggesting changes in the immunocharacteristics of lymphoma cells before and after delivery

BackgroundThe occurrence of malignant lymphoma after delivery is an extremely rare event. Although several cases of Hodgkin lymphoma and B cell lymphoma and a few cases of peripheral T cell lymphoma (PTCL) after delivery have been reported, there are no report of autopsy cases of PTCL in the puerperal period.Case presentationA 32-year-old Japanese woman with a past medical history of atopic dermatitis and bronchial asthma presented with generalized eruptions four days after the delivery of her first child; generalized skin induration and lymphadenopathy subsequently emerged. A skin biopsy specimen showed the diffuse proliferation of atypical lymphoid cells that were immunohistochemically-positive for CD4 but negative for CD8. She was diagnosed as PTCL, not otherwise specified (PTCL, NOS). She died one year and three months after the onset of symptoms. At autopsy, the systemic infiltration of lymphoma cells into the whole body was observed. Unexpectedly, these lymphoma cells were immuno-reactive with CD8 but not with CD4.ConclusionThe occurrence and development of PTCL after delivery with the shift from CD4 positivity to CD8 positivity may be associated with not only the selection of resistant subclone as a result of chemotherapy but also the changes of immune status before and after delivery.

Ganglion cyst in the temporomandibular joint: A case report and discussion of surgical approaches

Abstract Background: Ganglion cysts in the temporomandibular joint are treated by resection; however, there has been insufficient discussion regarding the appropriate approach for surgical resection. Clinical presentation: A 55-year-old man presented with a left preauricular swelling for one year. The examination revealed a moderately hard mass with a 30-mm diameter, tenderness, and restricted movement. Magnetic resonance imaging showed a cystic lesion with a 25 mm-long axis. Considering a diagnosis of temporomandibular cyst, separation of the cyst wall through a preauricular incision was attempted. However, the cyst ruptured due to strong adherence on the reverse side. Curettage followed by discectomy was performed. Histopathological diagnosis was of a ganglion cyst. Among 39 reported cases, 24 were excised via the preauricular approach, with 8 intraoperative cyst ruptures. Conclusion: The parotidectomy approach should be employed when the cyst is large, at a low position, or adherent to the surrounding tissue, in order to achieve reliable excision.

A case of rectal adenocarcinoma found in endocervical cytology

背景:子宮頸部擦過に直腸腺癌細胞をみた1例を経験した.細胞像を見直し, 免疫組織化学的に大腸腺癌, 体部腺癌, 頸部腺癌と比較検討した.症例:78歳, 女性.2, 3年前より不正出血を認めたが, 痔からの出血と思い放置していた.不正性器出血を認め, 重度貧血と子宮頸癌を指摘され入院となった.子宮頸部に4×3cmの腫瘍を認め, 子宮頸部生検, 頸部擦過にて腺癌の診断がなされた.その後, 6×5.3cm, type 2型の直腸癌が発見され, 腫瘍は膣から子宮頸部に直接進展しており, 肉眼所見より直腸癌の子宮頸部浸潤と診断された.結論:免疫組織化学的に, 大腸癌はcytokeratin 20 (CK20), carcinoembryonic antigen (CEA) が陽性を示すが, 体部, 頸部腺癌はcytokeratin 7, CA 125, estrogen receptor, progesteronereceptorの陽性傾向があり, 鑑別に有効であった.今回の頸部腫瘍は免疫染色にてCK20, CEA陽性であり直腸癌の浸潤であることが証明された.子宮頸部擦過に直腸腺癌細胞を認めることもあり, 鏡検に注意する必要があると思われる.