[A Case of Long-Term Survival with Tumor Resection and DOX plus DTIC Therapy for Desmoid Tumor after FAP Surgery].

The patient is a 22-year-old, female. She had a family history of familial adenomatous polyposis(FAP)and a prophylactic total colorectal resection was performed for FAP at age of 18. She presented with fever and abdominal distention and palpated a mass with tenderness in the right lower abdomen. Contrast-enhanced CT scan of the abdomen showed a heterogeneous contrast effect around the tumor margins. With the diagnosis of intra-abdominal desmoid tumor, a partial duodenal resection, small bowel mass resection, and right fallopian tube resection were performed along with the tumor, and an artificial anus was created with the jejunum. Contrast-enhanced CT scan of the abdomen 16 months after resection of desmoid tumor showed a 6.5 cm long desmoid tumor recurrence in the mesentery. She received 5 courses of doxorubicin (DOX)plus dacarbazine(DTIC)therapy followed by continued NSAIDs. Seven years after the operation, she has been able to maintain the shrinkage of the recurrent tumor and is still on medication. Long-term surveillance is necessary because of the possibility of the appearance of other associated lesions in the future.

[A Case of Right Hemicolectomy for Inflammatory Myofibroblastic Tumor Arising in the Right Transverse Colon].

The patient was a 21-year-old male. He presented with right lower abdominal pain and showed tenderness in the same area. An abdominal contrast-enhanced CT examination revealed a 45 mm tumor that continuously stained in the right transverse colon. The patient presented with tenderness and rebound tenderness. Due to a suspected submucosal tumor, laparotomy was performed and an elastic hard tumor of 5 cm in size was found on the serous membrane side of the right transverse colon. As malignancy could not be ruled out, a right hemicolectomy with lymph node dissection was performed. The pathological diagnosis was an inflammatory myofibroblastic tumor(IMT), characterized by the proliferation of spindle- shaped spindle-shaped fibroblast-like cells with inflammatory cell infiltration. As of 9 years post-surgery, there has been no recurrence. However, long-term surveillance is necessary.

[A Case of Surgery for Skin Metastasis of Papillary Thyroid Carcinoma in a Very Elderly Patient to Improve the Quality of Life].

The patient was a 100-year-old woman who underwent right lobe thyroidectomy for papillary thyroid cancer in another hospital around 1990. She developed local recurrence in 2010 and underwent anterior tracheal local recurrence resection in May 2017. Subsequently, metastases were confirmed in the lateral cervical lymph nodes, but the patient only received TSH suppression because of her advanced age. Multiple pulmonary metastases developed in November 2020 and supraclavicular lymph node metastases in July 2021, but the patient was under the care of a visiting physician on best supportive care. The patient was referred to our hospital in January 2022 due to the appearance of a cutaneous mass in the sternal incision area, which gradually increased in size. A well-mobile, well-defined, spherical mass was found in the sternotomy area. The maximum diameter was 19 mm. The cytological findings were consistent with cutaneous metastasis of papillary thyroid carcinoma. As the patient was elderly and had multiple pulmonary metastases, she was temporarily observed. However, by June 2022, the mass had increased from 19 mm to 33 mm with cutaneous discoloration. There was no tendency for multiple pulmonary metastases to grow during this period. The decision was made to operate in order to prevent a decline in quality of life due to self-destruction of the cutaneous metastasis. The tumor was removed under general anesthesia, including the cutaneous discoloration, and reconstructed with a rhomboid skin flap. Post-operatively, there was no local recurrence or significant increase in pulmonary metastases. It is suggested that resection of cutaneous metastasis of malignant tumors may be effective in preserving quality of life even in a 100-year-old elderly patient like the present case and should be considered as an indication.

Risk Factors for Predicting Lymph Node Metastasis in Submucosal Colorectal Cancer.

Objectives: The cornerstone of treating colorectal cancer (CRC) is generally a surgical resection with lymph node (LN) dissection. The tools for predicting lymph node metastasis (LNM) in submucosal (SM) CRC are useful to avoid unnecessary surgical resection. Methods: Retrospectively, we analyzed 526 consecutive patients with SM CRC who underwent surgical resection at the Osaka International Cancer Institute, Osaka University Hospital, and Minoh City Hospital, Japan, between 1984 and 2012. The Osaka International Cancer Institute group and the Osaka University Hospital group were randomly divided into a training set and a test set of 2:1. The prediction model was validated in Minoh City Hospital. Results: We partitioned patients using three risk factors involved in the presence or absence of LNM in SM CRC: lymphatic invasion (Ly), budding grade (BD) and the depth of submucosal invasion (DSI) (cut-off value 2789 µm) that were significantly different in the multivariate analysis. As a result, a predictive model of "LNM <5%" when "Ly negative and DSI <2789 µm" was evaluated. We similarly partitioned by DSI 3000 µm as easy-to-evaluate values in clinical use. We developed the additional model for predicting LNM is 1.05%, that is, LNM <5%, when there are "Ly negative and DSI <3000 µm." Conclusions: As a limitation, only patients who underwent surgical resection were included in this study. This predictive model could help clinicians and CRC patients decide on the additional surgery required after endoscopic resection.

[A Case of Malignant Rectal B Cell Lymphoma That Was Difficult to Differentiate from Rectal Neuroendocrine Tumor].

A 74-year-old woman with a chief complaint of hematochezia was admitted to our hospital. Colonoscopy revealed a 2 cm submucosal tumor with an erosion in the Rb of the rectum. Biopsy showed various inflammatory cell clusters, but no malignant findings. Thoracoabdominal CT and abdominal MRI showed no abnormal findings other than that of rectal wall thickening. Somatostatin receptor scintigraphy performed on suspected rectal NET showed no abnormal accumulation. A total of 3 biopsies were performed, but a definitive diagnosis was not confirmed, and transanal rectal mucosal resection was performed for diagnostic purposes. Immunohistochemistry showed CD20(+)and bcl-2(+), and the lesion was diagnosed as malignant rectal B cell lymphoma. For 1 year postoperatively, the patient showed no recurrence. For this case, biopsy often failed to confirm a definitive diagnosis on rectal submucosal tumors. If the tumor is >1 cm in diameter in a rectal NET, a rectal resection with lymph node dissection is required, and anal function may be impaired. Local tumor excision for diagnosis and treatment may be worthwhile in rectal submucosal tumors if pre-resection diagnosis with biopsy is difficult.

[A Case of Nivolumab Responding to Multiple Lymph Node Metastases during Multidisciplinary Treatment for Esophageal Cancer].

In nivolumab therapy for unresectable advanced esophageal cancer, there are a few cases that show a complete response, and long-term survival can be expected in such cases. Here, we report a case in which nivolumab had a complete response to multiple lymph node metastases during multidisciplinary treatment for esophageal cancer and survived for a relatively long period despite being elderly. Examination of complete response cases provides us with significant insights in considering the unexplained biomarkers of immune checkpoint inhibitors and treatment discontinuation during complete response.

Usefulness of Disposable Skin Biopsy Punch for Cervical Occlusion after Cervical Conization.

Cervical occlusion is a rare but serious complication after conization of the uterine cervix. We report on a 69-year-old woman with cervical intraepithelial neoplasia (CIN) 2, who successfully underwent follow-up biopsy of an occluded cervical canal after conization using a disposable skin biopsy punch. The disposable skin biopsy punch was inserted into a slight dimple, which was considered a uterine os, in the direction of the cervical canal, and a sample of tissue was obtained from the occluded portion. This procedure was performed without anesthesia and was completed uneventfully. Histological examination of the excised tissue revealed CIN 3. In addition, the procedure resulted in recanalization of the occluded cervical canal. The occlusion was limited in the external portion of the cervical os and attributed to adhesion of scar tissue during the healing process. Our experience suggests that a disposable skin biopsy punch can be used for follow-up biopsy and recanalization in patients with cervical occlusion after conization as a less invasive approach.

Robot-assisted laparoscopic surgery after placing a self-expanding metallic stent for malignant rectal obstruction: a case report.

BACKGROUND: Approximately 20% of colorectal cancer patients show complete or incomplete bowel obstruction as an early symptom. Preoperative nonsurgical decompression such as placing a self-expanding metallic stent for malignant colorectal obstruction has been shown to be effective for reducing perioperative morbidity and mortality. However, there is a lack of published studies reporting robot-assisted laparoscopic surgery (RALS) after self-expanding metallic stent (SEMS) placement for malignant rectal obstruction (MRO). To our knowledge, this is the first report to do so. CASE PRESENTATION: An 80-year-old man with incomplete paralysis of the lower limbs as well as bladder-rectal disorder due to a spine fracture sustained in a fall accident 26 years ago presented with lower abdominal pain and vomiting. Abdominal multi-detector computed tomography revealed an obstructive rectal tumor with distended bowel on the oral side. Emergency colonoscopy was performed, and an SEMS placed. The patency of SEMS and decompression of the distended bowel was confirmed, and elective RALS was performed 29 days after SEMS placement. To our knowledge, this is the first report of RALS after decompression with SEMS placement for MRO. CONCLUSIONS: RALS after SEMS placement is a safe and feasible therapeutic strategy for MRO.

Laparoscopic colectomy after self-expanding metallic stent placement through the ileocecal valve for right-sided malignant colonic obstruction: A case report.

A 78-year-old man with a history of open sigmoidectomy for sigmoid cancer presented with abdominal pain and vomiting. Abdominal multi-detector CT revealed an obstructive ileocecal tumor with distended small bowel on the oral side. We performed emergency drainage using a transnasal decompression tube, and 2 days later, we conducted a colonoscopic examination, which lead to a provisional diagnosis of obstruction with a malignant tumor invading the ileocecal valve. We then placed a self-expanding metallic stent (SEMS) through the ileocecal valve. We confirmed patency of the ileocecal valve and removed the transnasal decompression tube 2 days after SEMS placement. We then performed elective laparoscopic colectomy 8 days after SEMS placement. To the best of our knowledge, there has been only one previous report of laparoscopic colectomy after decompression with SEMS placement through the ileocecal valve for right-sided malignant colonic obstruction.

Unusual False-Positive Mesenteric Lymph Nodes Detected by PET/CT in a Metastatic Survey of Lung Cancer.

Positron emission tomography/computed tomography (PET/CT) is a credible diagnostic modality for detecting primary and metastatic malignancy. PET/CT sometimes shows false positives and negatives, which make clinical diagnosis difficult. A 42-year-old man who had undergone right upper lobectomy for lung cancer 1 year previously had PET/CT for a metastatic survey of the lung. The lung cancer was stage IB (pT2N0M0) bronchioloalveolar carcinoma. PET/CT showed massive (18)F-fluorodeoxyglucose (FDG) uptake in the mesenteric lymph nodes. Because the mesentery is an unusual site of metastasis, the patient was under watchful observation. Another PET/CT after 6 months still showed FDG uptake in the same location, with a slightly increased standard uptake value. A systemic survey was performed, but it did not reveal any malignancies or inflammatory diseases. Eventually, the patient underwent probing laparoscopic surgery. For complete resection of the lymph nodes, laparoscopic ileocecal resection was performed. Histologically, the resected lymph nodes showed reactive lymphadenitis. Glucose transporter 1 immunostainings of the lung cancer and the lymph node were positive and partially positive, respectively. Although PET/CT is a powerful diagnostic modality, clinical interpretation of unusual results is difficult.

[A Case of Angioimmunoblastic T-Cell Lymphoma Merged with Colorectal Cancer That We Were Able to Resect after a Chemotherapy Response].

The patient was 77-year-old man. He visited our hospital with the chief complaint of an abdominal mass in March 2015. We diagnosed the patient with transverse colon cancer and he was suspected of having malignant lymphoma. In March 2016, we attempted to perform right hemicolectomy for the transverse colon cancer, but it was difficult because swollen lymph nodes had formed a large mass with the surrounding tissue, including vessels of the mesentery. We could only complete the surgery after mesenteric lymph node biopsy. On the basis of the results of this biopsy, we diagnosed angioimmunoblastic Tcell lymphoma. At first, we administered THP-COP therapy for malignant lymphoma. However, after 3 courses of this therapy, the patient suddenly developed ileus due to the aforementioned colon cancer. According to enhanced CT performed at the onset of intestinal obstruction, chemotherapy dramatically reduced the size of the intraperitoneal lymph nodes. We therefore concluded that it was able to treat the colon cancer. We performed right hemicolectomy in June 2015. Angioimmunoblastic T-cell lymphoma comprises only 2-3% of all malignant lymphoma cases. We experienced a rare case of complications from angioimmunoblastic T-cell lymphoma and colorectal cancer.

[Successful Treatment of Local Recurrence of Advanced Gastric Cancer Using Curative Gastrectomy via Distal Pancreatectomy after Chemoradiotherapy].

The patient was a 65-year-old woman. She was diagnosed with advanced gastric cancer with liver invasion. After receiving systemic chemotherapy(S-1 plus PTX)for 3 months, she underwent total gastrectomy and partial hepatectomy in May 2008. Because she developed celiac artery circumference lymph node recurrence in November 2010 during postoperative adjuvant chemotherapy consisting of S-1 plus PTX, we changed her chemotherapy regimen to CPT-11 plus CDDP. We observed an increase in the size of the lymph nodes in August 2013 and the response was poor even after switching to DOC. However, the lymph nodes continued to increase in size and we administered radiotherapy of 60.4 Gy/33 Fr that resulted in shrinkage of the nodes. We observed an increase in lymph node size and pancreas invasion in September 2015, including an expansion of the mid pancreatic duct. We performed distal pancreatectomy without identifying the recurrence observed in November 2015 assuming it was an exacerbation. Six months after the surgery, the recurrence was not apparent. We report an example of long-term survival that was achieved for Stage IV gastric cancer. The patient underwent combined modality therapy for 8 years, and local recurrence was controlled via a primary operation.

[Two Cases of Small Intestinal Metastasis of Lung Cancer].

Case 1: A 66-year-old man who had undergone upper right lobe resection for large cell lung carcinoma 2 months earlier presented with abdominal pain and vomiting. Computed tomography showed intussusception of the small intestine. Partial resection of the small intestine was performed. The tumor was pathologically diagnosed as lung cancer metastasis to the small intestine. The patient died 30 days after surgery. Case 2: A 57-year-old man was admitted to hospital due to appetite loss. Computed tomography showed a large intestinal tumor and a small lung tumor, as well as multiple liver metastases and lymph node metastases. We diagnosed perforation of the small intestine tumor, and partial resection of small intestine was performed. Pathological examination and immunohistochemical staining revealed that the tumor was a metastatic adenocarcinoma, and the patient was diagnosed with small intestine metastasis of lung cancer. He died 75 days after surgery. Although small intestine metastasis of lung cancer is rare, the incidence is increasing. The prognosis of lung cancer metastasis of the small intestine is poor, and therefore, we must urgently decide the appropriate treatment.

[Renal Epithelioid Angiomyolipoma with Spontaneous Rupture: A Case Report].

A 66-year-old woman was referred to our hospital for a right renal mass found in the examination for sudden right flank pain. Abdominal computed tomography (CT) revealed a right renal tumor, 8.0 cm in diameter, with massive hemorrhage due to spontaneous tumor rupture. After transcatheter arterial embolization, right radical nephrectomy was successfully performed. The histopathological diagnosis of the renal tumor was epithelioid angiomyolipoma (eAML). Postoperative chest CT showed two lung tumors. Therefore, the lung tumors were resected and diagnosed as a primary lung adenocarcinoma and a sclerosing angioma. Although renal eAML is thought to have malignant potential, there has been no local reccurence nor distant metastases of renal eAML 11 months after the surgery.

[A Case in Which a Patient Was Operated for Intra-Abdominal Desmoid Tumors after Total Colectomy in FAP].

The patient was a 22-year-old woman with FAP, who had undergone laparoscopic total colectomy 3 years previously. She presented to our hospital with a high fever and abdominal pain. Large hard tumors were palpated in the right lower abdomen and pelvis. Blood examination showed an inflammatory response. CT scan revealed 17 cm diameter solid tumors. At surgery, 2 tumors arising from the mesentery of the small intestine were found, neither of which invaded any organs. We performed tumor extirpation with partial resection of the duodenum, ileum, right fallopian tube and rectum. A jejunal stoma was formed, leaving a length of remnant intestine of about 120 cm. The histopathological diagnosis was given as desmoid tumor with infection. The patient was discharged from the hospital on the 9th postoperative day. Desmoid tumor is the second most common cause of death in FAP patients. Although desmoids can also occur in the extremities, most FAP patients develop intra-abdominal tumors. Despite being histologically benign, they are locally infiltrative and can cause death through invasion and destruction of adjacent vital structures and organs. Here, we report a case of desmoid tumors with FAP with reference to the literature.

[Intraluminal Type Gastrointestinal Stromal Tumor Resected by Single-Incision Percutaneous Endoscopic Intragastric Surgery--A Case Report].

We report a patient with gastric gastrointestinal stromal tumor (GIST) who underwent single-incision percutaneous endoscopic intragastric surgery. The patient was a 70-year-old man. Esophagogastroduodenoscopy and abdominal computed tomography revealed the presence of an intraluminal type gastric submucosal tumor, 4 cm in diameter, in the posterior wall of the gastric body. Laparoscopic partial gastrectomy was performed via a single incision made in the epigastric region. The postoperative course was uneventful. The pathological diagnosis was a low-risk GIST. This method is easy and safe to perform; therefore, we consider it to be an important option for the treatment of intraluminal type gastric GIST.

Localized donor cells in brain of a Hunter disease patient after cord blood stem cell transplantation.

The efficacy of hematopoietic stem cell transplantation (HSCT) for Hunter disease (deficiency of iduronate-2-sulfatase, IDS) remains unclear. We treated a 6-year-old male suffering from a severe type of Hunter disease with cord blood stem cell transplantation (CBSCT); however, he died at 10 months post-therapy due to a laryngeal post-transplantation lymphoproliferative disorder. During the follow-up period after CBSCT, his hyperactivity, estimated mental age, and brain MR findings had not improved. We assessed the efficacy of CBSCT by biochemical and pathological analyses of the autopsied tissues. There were many distended cells with accumulated substrate in the brain, but not in the liver. IDS enzyme activity in the cerebrum remained very low, although that in the liver reached about 40% of the normal control level. However, a variable number of tandem repeats analyses demonstrated a weak donor-derived band not only in the liver but also in the cerebrum. Furthermore, IDS-immunoreactivity in the liver was recognized broadly not only in Kupffer cells but also in hepatocytes. On the other hand, IDS-immunoreactivity was recognized exclusively in CD68-positive microglia/monocytes in the patient's brain; whereas that in the normal brain was also detected in neurons and oligodendrocytes. These donor-derived IDS-positive cells were predominantly localized in perivascular spaces and some of them were evidently present in the brain parenchyma. The efficacy of CBSCT was judged to be insufficient for the brain at 10 months post-therapy. However, the pathological detection of donor-derived cells in the brain parenchyma suggests the potential of HSCT for treatment of neurological symptoms in Hunter disease. This is the first neuropathological report documenting the distribution of donor-derived cells in the brain after CBSCT into a Hunter disease patient.

Intravascular lymphomatosis initially suspected from uterine cytology: a case report.

BACKGROUND: Because recognizable lesions are often absent, selection of biopsy sites for diagnosis of intravascular large B-cell lymphoma (IVL) is frequently problematic. CASE: A 59-year-old woman was admitted with fever and general fatigue. Combined physical and roentgenographic examinations revealed neither lymphadenopathy, hepatosplenomegaly nor mass lesions in other organs. Serum lactate dehydrogenase level was 1412 IU/L. There were no genital symptoms, but uterine cytologic examination revealed large cells distributed in a noncohesive pattern. These cells had a large, irregularly shaped nucleus in which several nucleoli were discernible and showed positive immunoreactivity for leukocyte common antigen. Three months after admission, neurologic symptoms appeared, and magnetic resonance imaging revealed multiple nodular lesions in the brain. Biopsy specimens from the brain lesion showed the proliferation of large lymphoid cells filling the lumina of small vessels and Virchow-Robin's space. Immunohistochemistry revealed that the tumor cells were positive for CD20 and CD79a but negative for CD3, indicative of IVL. CONCLUSION: Uterine cytologic and/or histologic examinations could be the choice for diagnosis of IVL, even when genital symptoms are absent.

[A case of gastrointestinal stromal tumor of the stomach with rapid growth in a short term].

A 75-year-old man was admitted to our hospital with massive hematemesis. Upper gastrointestinal endoscopy revealed a submucosal gastric tumor with ulcer lesion, about 30 mm in diameter, in the anterior wall of the cardia. Pathological findings of biopsy specimens showed a gastrointestinal stromal tumor (GIST) of the stomach. Abdominal computed tomography (CT) showed an extramural low density mass, measuring 27 mm, in the cardia. From these findings, we thought we should be performing a wedge resection of the stomach for this tumor. After one month from the first examination, preoperative upper gastrointestinal endoscopy revealed a growth of the tumor. Abdominal CT also showed an obvious growth of the tumor, measuring 50 mm. Thus, laparoscopy-assisted proximal gastrectomy was performed, because the tumor was close to the cardia. Postoperative diagnosis of the tumor was pathologically confirmed to be a GIST with a high malignant potential. The case report of GIST that shows rapid growth for a short term is rare. We herein describe a case of GIST with rapid growth during one month before the operation.