Situs Inversus Totalis with Malignant Lymphoma of the Stomach: Report of a Case

Situs inversus totalis is a rare congenital anomaly that often occurs concomitantly with other disorders. We report a case of situs inversus totalis with malignant lymphoma of the stomach, which was successfully treated by surgery followed by chemotherapy and irradiation. The patient was a 51-year-old woman who present with colicky pain in the left upper quadrant of her abdomen. Chest X-ray showed a right-sided heart, and ultrasonography and computed tomography (CT) of the abdomen showed a situs inversus totalis with multiple gallstones in the gallbladder. Tree-dimensional reconstructed CT of the abdomen showed no other malformations coexisting with situs inversus totalis, but a barium upper gastrointestinal series found an inverted stomach and an elevated tumor with ulceration in the center, localized in the antrum of the stomach. First, we performed a cholecystectomy, followed by a total gastrectomy with dissection of the lymph nodes and splenectomy, and Roux-en-Y reconstruction. Histopathological examination confirmed a diagnosis of malignant lymphoma of the stomach (diffuse large B-cell type) with metastasis to the regional lymph nodes. Chemotherapy using the CHOP regimen was given three times, starting 1 month postoperatively. A follow-up CT scan showed enlargement of one lymph node around the abdominal aorta and irradiation was delivered to the area of the inverted Y in the abdomen. At the time of writing, 10 months after surgery, the patient is well with no signs of recurrence and leading a normal life. Careful preoperative assessment is very important for determining the most appropriate surgical procedure in patients with situs inversus totalis associated with a malignancy. Received: April 25, 2002 / Accepted: November 19, 2002 RID="*" ID="*" Reprint requests to: S. Murakami     

Complete Cure of Malignant Lymphoma of the Stomach with a Huge Adrenal Lesion Achieved by Preoperative Chemotherapy and Surgery: Report of a Case

We report herein the case of a 53-year-old woman with malignant lymphoma of the stomach who was successfully treated by preoperative chemotherapy and surgery. The patient consulted our hospital with the chief complaint of upper abdominal pain. Endoscopy demonstrated a protruding lesion at the antral posterior wall of the stomach, and a post-biopsy pathological diagnosis of diffuse large cell type B-cell lymphoma was established. Moreover, abdominal ultrasonography, computed tomography, and magnetic resonance imaging demonstrated a tumor measuring approximately 10 cm in diameter in the left adrenal gland. A total of three courses of chemotherapy using the CHOP regimen were given preoperatively. The CHOP regimen consisted of 100 mg of prednisolone administered for 8 days together with 1.9 mg of vincristine, 1 000 mg of cyclophosphamide, and 60 mg of epirubicin administered intravenously on the first day. This resulted in tumor shrinkage, and a distal gastrectomy, lymph node dissection, and left adrenalectomy were subsequently performed. Since the pathological findings of the resected tissue specimen demonstrated complete elimination of the malignant lymphoma, this combination of procedures was defined as having resulted in a complete response. The postoperative course of this patient was uneventful. She is still alive without any sign of tumor recurrence 6 years after her operation, and is being followed up at the outpatient clinic. Received: September 27, 1999 / Accepted: September 26, 2000     

Pleomorphic Carcinoma of the Lung with Mediastinal Extension Following Malignant Lymphoma: Report of a Case

In following up a patient with non-Hodgkin's lymphoma, we encountered a case of pulmonary pleomorphic carcinoma with mediastinal direct invasion. A 65-year-old man with hemoptysis was found to have an abnormal shadow in the right upper lung field. A 6.4 × 4.8-cm tumor adjacent to the upper mediastinum occupied the right anterior segment of the upper lobe (S3) and invaded the superior vena cava (SVC). The serum level of neuron-specific enolase was elevated to 11.9 ng/ml. A specimen from a transbronchial lung biopsy of the right B3b bronchus revealed giant tumor cells. A right upper lobectomy with SVC reconstruction was performed. The resected tumor was diagnosed as a pulmonary pleomorphic carcinoma with a large component of giant and spindle cells, and it is considered to be a rare histologic type.     

Primary Malignant Mesothelioma of the Greater Omentum: Report of a Case

We report a rare case of primary malignant mesothelioma of the greater omentum. To our knowledge, only one other such case has been described in the English literature. The patient was a 61-year-old Taiwanese woman without any history of exposure to asbestos, who presented with lower back pain. Abdominal sonography and computed tomography showed a 12 × 9 × 9-cm³ mass occupying the lower abdomen. Laparotomy revealed a tumor in the greater omentum, invading the posterior wall of the uterus, without diffuse mesenteric thickening or multiple small nodules in the peritoneum. We performed en bloc resection of the mass, which involved omentectomy, hysterectomy, and bilateral salpingo-oophorectomy. Microscopically, the tumor cells were arranged in a tubulopapillary pattern lined by a single layer of uniform, cuboidal cells. A pattern of sclerotic stroma with irregular glandular elements was also recognized. Immunohistochemically, the tumor cells showed strong positivity for calretinin. The final pathologic diagnosis was malignant mesothelioma. The patient did not receive chemotherapy or radiotherapy, and has remained in good health without any evidence of recurrence for almost 3 years since her operation.     

Malignant Sarcomatoid Tumor of the Liver: Report of a Case

A 65-year-old man was referred to our hospital for treatment of a liver tumor. Abdominal ultrasonography (US) demonstrated a low echoic mass in the S2–S4 region of the liver, which was confirmed by abdominal computed tomography (CT). In the delayed phase of angio-CT, the inside of the mass was not enhanced. Abdominal angiography showed a hypovascular area in the liver. An extended left lobectomy was performed. Macroscopically, the tumor was 9.5 × 9.5 cm in size, and on cross section, it was white and clearly demarcated from the surrounding tissue. Microscopic observation of hematoxylin–eosin-stained specimens did not show any glandular or trabecular formation. Histologically, there was diffuse proliferation of atypical spindle cells that had hyperchromatic, short, spindle-shaped nuclei, and pale cytoplasm with poor intercellular adhesion. The nontumorous tissue was almost normal with no sign of cirrhosis. Immunohistochemical examination showed that the spindle cells were positive for vimentin and cytokeratins (AE1/AE3, CAM 5.2), but negative for all other markers. The final diagnosis was a sarcomatoid carcinoma, the origin of which was not able to be confirmed immunohistochemically. This case of a primary hepatic tumor composed of malignant cells with sarcomatous features is described, and the immunohistochemical findings are discussed. Received: February 21, 2000 / Accepted: July 25, 2000     

Adenocarcinoma Within a Paracoccygeal Teratoma in an Adult: Report of a Case

We report an unusual case of adenocarcinomatous transformation of a paracoccygeal teratoma (PCT) in a 44-year-old woman. The patient was referred to us for surgical treatment of a growing paracoccygeal mass. The findings of a magnetic resonance imaging scan were compatible with soft tissue malignancy. Using the posterior approach, we performed complete surgical removal with coccygectomy. Histological examination of the cystic mass revealed a mature teratoma containing primary adenocarcinoma. The patient has been followed up for 7 years and is free of disease. Because prognosis depends on complete excision, we emphasize the importance of clear-margin surgery, including en bloc removal of the coccyx.     

Gastrointestinal Stromal Tumor of the Stomach: Report of a Case

We report herein the case of a 70-year-old woman found to have a gastrointestinal stromal tumor (GIST) of the stomach. Preoperative X-ray and endoscopic examination revealed a hemispheric submucosal tumor with central depression in the anterior wall of the gastric fornix. The tumor, which was 3 cm in diameter, was resected by a laparoscopy-assisted procedure. Histologic examination revealed that it was composed of spindle-shaped cells with elongated nuclei, and few mitoses. Most of the tumor cells showed immunoreactivity for vimentin and CD34, but not for α-smooth muscle actin, desmin, or S-100 protein. The PCNA index was 40.5%. Thus, the GIST did not show differentiation toward smooth muscle or neural cells. A gastrectomy was not performed because the small size of the tumor, and the paucity of the mitoses indicated that it was benign. Nevertheless, careful and long-term follow-up is needed to monitor for signs of possible local recurrence or distant metastases. Received: February 9, 2000 / Accepted: September 26, 2000     

Pancreaticoduodenectomy for Perforated Gastric Malignant Lymphoma: Report of a Case

We herein report the case of a 65-year old man with gastroduodenal malignant lymphoma (diffuse large B-cell type) accompanied with a bulbar perforation. A pancreaticoduodenectomy was performed because the duodenal second portion and pancreatic head were involved. However, extensive lymphadenectomy was not carried out because many lymph nodes were involved. The postoperative course was uneventful and CHOP therapy (pirarubicin 30 mg/m², cyclophosphamide 500 mg/m², vincristine 1 mg/m², prednisolone 30 mg/m²) was started 1 month after surgery. Seven cycles of CHOP therapy markedly reduced the residual lesions including lymph nodes, and a computed tomography scan after the final chemotherapy revealed no obvious lesions. Complete remission was obtained and the patient was discharged 9 months after surgery. Received: October 18, 2000 / Accepted: November 20, 2001     

Malignant Mixed Mullerian Tumor of the Ovary Growing into an Inguinal Hernia Sac: Report of a Case

Malignant tumors presenting as an inguinal hernia are rare. We present the case of a malignant mixed Mullerian tumor (MMMT) of the ovary growing into an inguinal hernia sac. In this case, magnetic resonance imaging was useful in making a diagnosis of an ovarian neoplasm growing into the inguinal canal, and to the best of our knowledge, this is only the tenth case of a malignant ovarian tumor and the first case reported in the English-language literature of MMMT of an ovary which grew into an inguinal hernia sac.     

Successful Treatment of Multiple Small-Bowel Perforations Caused by Cytomegalovirus in a Patient with Malignant Lymphoma: Report of a Case

We report the successful management of multiple small-bowel perforations caused by cytomegalovirus (CMV) infection in a 60-year-old man, 1 day after CHOP (cyclophosphamide/doxorubicin/vincristine/prednisone) therapy induction for malignant lymphoma. Emergency laparotomy was performed for perforative peritonitis, but we did not resect the lesions at this time. Instead, we exteriorized the small bowel and then irrigated the peritoneal cavity and intestinal tract. His white blood cell count was low, at 200 cells/μl, so this therapy was continued until it recovered. The intestine was highly edematous, but it improved after irrigation with peritoneal dialysis solution. In the second-stage procedure, we resected the small bowel with the perforations, and constructed a jejunostomy and colostomy, then closed the abdominal cavity. Although the patient needed central venous hyperalimentation, he had a favorable postoperative course and started treatment again for the malignant lymphoma.     

Poorly Differentiated Adenocarcinoma with Signet-Ring Cell Carcinoma in a Hyperplastic Polyp of the Stomach: Report of a Case

Hyperplastic polyps (HPs) of the stomach have been reported to be mostly benign. However, in rare cases, carcinomas have been found in HPs. We treated a 59-year-old Japanese male who underwent a total gastrectomy, and a gross examination of the resected stomach revealed a 4.8 × 3.8-cm polyp on the greater curvature of the antrum and multiple small polyps in the whole gastric mucosa. Histologically, the large polyp consisted mainly of hyperplastic foveolar epithelium, while the presence of variously colored lobules demonstrated a poorly differentiated adenocarcinoma mixed with signet-ring cell carcinoma. Hyperplastic polyps should therefore be carefully examined microscopically as a polypectomy specimen and in resected stomach specimens.     

Mucin-Producing Tumor of the Pancreas Associated with Pyothorax: Report of a Case

We report herein the case of a 76-year-old man for whom an invasive mucin-producing tumor of the pancreas (MPTP) was successfully treated by surgery. A cystic lesion of the pancreas had been found by computed tomography (CT) 9 years earlier, 2 years following which suction drainage for left pyothorax had been carried out. A pancreatic cyst fistula to the thorax had subsequently been found during decortication for recurrent pyothorax 2 years later. Methicillin-resistant Staphylococcus aureus was detected in the pleural discharge after the thoracotomy, and thoracic fenestration was performed. A CT scan done 4 years later showed enlargement of the pancreatic cysts and a cystography revealed communication to the duodenum via the main pancreatic duct. Endoscopic retrograde cholangio-pancreatography (ERCP) showed dilatation of the main pancreatic duct. The pancreatic cyst fistulated to the stomach and to the fenestrated thorax. Since MPTP was suspected from this clinical course, a distal pancreatectomy, partial gastrectomy, and omentopexy to the thorax were performed. The pathological diagnosis was intraductal papillary-mucinous tumor of the pancreas with a megacyst. While MPTP is recognized as a low-grade malignancy, some cases of invasive disease have been reported. To the best of our knowledge, this is the first case of MPTP associated with pyothorax due to fistula formation. Received: February 21, 2000 / Accepted: November 20, 2000     

Malignant Fibrous Histiocytoma of the Abdominal Cavity: Report of a Case

Malignant fibrous histiocytoma (MFH) is a soft-tissue sarcoma originating from fibroblast cells, characterized by a high rate of metastasis or recurrence. This tumor rarely develops in the gastrointestinal tract, with no more than 30 cases described in the literature. We report a case of MFH of the abdominal cavity in a 45-year-old woman who presented with epigastric pain, anorexia, and weight loss. A computed tomography (CT) scan of the abdomen revealed multiple solid tumors in the peritoneal cavity. We performed exploratory laparotomy and found at least 15 solid whitish tumors attached to the wall of the small intestine, as well as to the parietal peritoneum. There were three metastases in the liver. All of the tumors were excised, most of which were about 10cm in diameter. Histopathological findings indicated a stromal tumor consisting of spindle cells, and immunohistochemical examination of the resected specimens established the definite diagnosis of a pleomorphic MFH. The patient had an uneventful postoperative course and was given adjuvant chemotherapy. She is currently well 2 years after her operation. We review the clinical picture of this tumor in the abdominal cavity, and discuss its diagnosis, pathogenesis, and treatment.     

Successful Treatment of Malignant Fibrous Histiocytoma Originating in the Chest Wall: Report of a Case

Malignant fibrous histiocytoma (MFH) rarely originates in the chest wall, so its clinical features are not well defined. We report a case of MFH that recurred locally 3 years after primary resection. The patient, a 59-year-old woman, underwent wide excision, and is alive without recurrence 7 months after the operation. We reviewed the clinical features and treatment strategies of the total 39 cases of MFH originating in the chest wall reported from Japan. The fact that all patients who underwent wide excision with negative margins at the primary operation were alive without recurrence at the time of each report, despite a local recurrence rate as high as 40%, shows the importance of this operative strategy. Thus, early diagnosis of MFH of the chest wall is essential for improving the outcome of these patients. Neoadjuvant chemotherapy plus radiation therapy may be worthwhile for patients with advanced disease.     

Primary Lymphoma of the Liver: Report of a Case

(Received for publication on Sept. 11, 1998; accepted on Nov. 11, 1999)     

Surgical Treatment of Malignant Fibrous Histiocytoma in the Left Atrium and Pulmonary Veins: Report of a Case

This report describes the case of a 16-year-old boy who underwent surgical treatment of a cardiac malignant fibrous histiocytoma (MFH). He was admitted to our hospital for exertional dyspnea. An ultracardiogram (UCG) revealed a tumor about 10 cm in diameter, in the left atrium. Extirpation of the left atrial tumor, including the part extending into the pulmonary veins, was performed under cardiopulmonary bypass. Histological examination confirmed a diagnosis of MFH. No adjuvant chemotherapy or radiotherapy was given. While there has been no evidence of local recurrence or metastasis in the 9 months since his operation, strict follow-up is being done by UCG and computed tomography. A few reviews of this entity have been reported; however, they have confused autopsy cases with surgical cases. Therefore, we reviewed only the surgical cases of this type of cardiac tumor documented in the literature. According to our review of the literature, this patient is the youngest among the 42 cases of surgically treated cardiac MFH reported to date. Received: December 28, 2000 / Accepted: September 11, 2001     

Hodgkin Lymphoma of the Nasopharynx: Case Report with Review of the Literature

We report an uncommon case and histopathologic work-up of Hodgkin lymphoma of the nasopharynx in a 49-year old female patient who presented with a 2-year complaint of bilateral nasal congestion. Histologic study revealed a lymphocyte rich subtype of classic Hodgkin lymphoma. Immunohistochemical analysis revealed CD15, CD30, OCT-2, BOB.1, and MUM-1 expression by the neoplastic cells and a lack of expression of CD45, CD20, CD3, EMA, and EBER. The review of the literature showed that Hodgkin lymphoma of the nasopharynx is rare, the most common reported subtype is the mixed cellularity, and Hodgkin lymphoma of the nasopharynx has a favorable prognosis.     

Alpha-Fetoprotein-Producing Esophageal Adenocarcinoma: Report of a Case

Alpha-fetoprotein (AFP)-producing esophageal tumors are extremely rare. We report herein the case of a 51-year-old man found to have an AFP-producing adenocarcinoma arising from esophageal proper mucosa. The patient presented for investigation of dysphagia, and esophagogram and endoscopy revealed a lesion about 2 cm in size with a depressed center surrounded by low nodular protrusions in the lower esophagus. The preoperative serum AFP concentration was elevated to 52.4 ng/ml. A subtotal esophagectomy was performed, and macroscopic examination of the resected specimen revealed a superficial protruding lesion. Histopathological studies showed a poorly differentiated adenocarcinoma with a single lymph node metastasis. The tumor had infiltrated the submucosal layer, but there was no evidence of lymphatic or venous invasion. Immunohistochemical study revealed tumor cells positive for AFP. There were no findings of Barrett's epithelium or any mucosal changes due to reflux esophagitis. An elevated AFP level 2 years after the operation led us to suspect tumor recurrence; however, diagnostic imaging studies showed no evidence of a recurrence or metastases. The serum AFP levels responded well to chemotherapy with transient decreased levels, but continued to rise until finally, 5 years after the operation, adenocarcinoma cells were detected in the pleural effusion. Thus, careful monitoring of the serum AFP levels at regular intervals could be a useful marker to indicate recurrence of esophageal carcinoma. Received: September 7, 2000 / Accepted: May 15, 2001     

Malignant Granular Cell Tumor of the Esophagus: A Case Report

Introduction: Malignant granular cell tumor (MGCT) of the esophagus is an extremely rare malignancy with a poor prognosis. Literature describing this condition is not sufficient, especially regarding long-term survival.Presentation of Case: A 52-year-old woman presented with dyspnea and slow onset dysphagia. The endoscopy, endoscopic ultrasound (EUS), bronchoscopy, and positron emission tomography (PET)/computed tomography (CT) supported the suspicion of esophageal gastrointestinal stromal tumor (GIST). Open wedge esophagectomy and tracheal resection were performed. The histology proved periodic acid–Schiff (PAS)-positive granules in epithelial cells, hyperchromatic nuclei and the positivity of Protein soluble in 100% ammonium sulfate (S-100), vimentin, neuron-specific enolase, laminin, and myelinic proteins. Local recurrence after 10 months required a two-phase esophagectomy with retrosternal gastroplasty. Bone, liver, and mediastinal metastases occurred 6 months later, with overall survival of 34 months.Discussion: Preoperative histological confirmation is often not reliable. Tracheal invasion increases the perioperative risk and the probability of an unsuccessful resection. Esophagectomy or radical R0 local resection is the only known curative therapy. Repeated resections may increase survival in case of locoregional recurrence. Radiotherapy has a potential for palliative care.Conclusion: Esophageal MGCT requires a detailed presentation including long-term survival. Early surgical removal of intramural esophageal neoplasms with potentially malignant features is highly recommended. Radical and/or repeated esophageal resections are the only known therapies with curative potential.     

A Metastatic Ball Tumor in the Right Atrium Originating from Esophageal Cancer: Report of a Case

A metastatic ball tumor in the right atrium originating from esophageal cancer is extremely rare. A 53-year-old woman had two such tumors without any other types of metastasis. These tumors were associated with severe thrombocytopenia caused by the consumption of platelets. Even after repeated transfusions of platelets and fresh frozen plasma, the thrombocytopenia could not be controlled. Therefore, a surgical resection was performed to improve the thrombocytopenia and avoid sudden death due to a complete obstruction of the right ventricular outflow tract by the tumor. As the throm-bocytopenia did not recur postoperatively, the patient was discharged from hospital. She died, however, of multiple metastases with bilateral pleural effusions 5 months after the surgery.