Neuroendocrine Tumor Arising within Mature Cystic Teratoma of the Pancreas: Literature Review and Case Report

Cystic teratomas are germ cell tumors most commonly found in the ovaries and testes. The pancreas, however, is very rare as a site of occurrence. Moreover, only two cases of cystic teratoma with concomitant neuroendocrine tumor have been reported to date. We report the case of a 33-year-old female who presented with abdominal pain. Computed tomography and magnetic resonance imaging of the upper abdomen revealed an 85 mm cystic tumor in the head of the pancreas. Cystic teratoma and mucinous cystadenoma were suggested as differential diagnoses. Cytopathologic analysis of endoscopic ultrasound-guided fine needle aspiration was consistent with mucinous cystadenoma. Therefore, the patient underwent surgical resection. Histologic analysis revealed a mature cystic teratoma of the pancreas with a concomitant neuroendocrine tumor. The patient is in great condition at 8 months follow-up. Cystic teratoma of the pancreas with a concomitant neuroendocrine tumor is an extremely rare condition. Surgical resection remains the mainstay of treatment as it provides a definitive diagnosis and no recurrences have been reported to date.     

Case of a 23-year-old woman with serous surface papilloma of borderline malignancy of the left ovary and mucinous cystadenoma of the right ovary

A rare case is reported of a 23-year-old woman with serous surface papilloma of borderline malignancy (SSPBM) of the left ovary associated with a foci of peritoneal “implant,” as well as the coexistence of mucinous cystadenoma of the right ovary. The histologic diagnosis of these tumors was classified according to the criteria of the World Health Organization. To confirm the histologic diagnosis, special staining was performed both with AIcian blue, periodic acid-Schiff (PAS), and PAS with diastase digestion, and with immunohistochemical staining for vimentin, cytokeratin, epithelial membrane antigen (EMA), neuron-specific enolase, and carbohydrate antigen 19-9 (CA 19-9). After hematoxylin and eosin staining of the tissue samples, the right ovarian tumor was diagnosed as benign mucinous cystadenoma, and the left ovarian tumor was identified as serous surface papilloma with no apparent stromal invasion, but showing proliferating activity and nuclear abnormality (SSPBM). The histologic features of the peritoneal lesion resembled those of the SSPBM of the left ovary, and showed no invasion of underlying normal tissue. Moreover, both the SSPBM and the peritoneal lesion showed similar patterns on special staining. The summary diagnosis was SSPBM of the left ovary associated with a foci of peritoneal noninvasive “implant” and coexistent mucinous cystadenoma of the right ovary. Regardless of whether or not the peritoneal loci represented an autochthonous multicentric tumor of the peritoneum or a secondary implant from the left SSPBM, the less aggressive histologic character of her tumors has allowed this patient to survive for 8 years after fertility-retaining surgery with no sign of recurrence.     

Ductectatic-type Mucinous Cystadenoma and Cystadenocarcinoma of the Human Pancreas: A Novel Clinicopathological Entity

A new type of mucinous cystic pancreatic neoplasm is described. The lesions, which have been recognized only recently with the progress of diagnostic techniques and have not previously been described in the literature, are characterized by multilocular cysts with papillary proliferation of the lining epithelium. They occur exclusively in the head and body, predominantly in males, and coexistence of well-differentiated adenocarcinoma as well as adenoma components is frequently encountered. These lesions are quite different from hitherto described mucinous cystic neoplasms of pancreas, not only in terms of gross features but also with regard to intrapancreatic location, and sex and age distributions. We propose to classify these lesions as a new pancreatic tumor entity: the ductectatic-type mucinous cystadenoma/cystadenocarcinoma.     

A mucin-producing cystadenoma, borderline malignancy, of the renal pelvis and ureter: a case report

A cystadenoma or a cystadenocarcinoma arising from the renal pelvis is a rare neoplasma and, so far as is known, only two cases have been reported in the literature (Arcadi, 1956, and Ross, 1985). Discussed herein is a case of a 63-year-old woman who developed a mucinous nephrosis, due to a marked retention of mucin produced by a tumor. The ureter as well was found to be involved. The histogenesis of an adenocarcinoma, which includes a cystadenoma and a cystadenocarcinoma, most likely is due to a glandular metaplasia, associated with urolithiasis and pyelonephritis. In this specific case, the patient revealed a history, fifteen years earlier, of renal calculi and pyelonephritis.     

Laparoscopic Diagnosis of Annular Pancreas in a Patient with Mucinous Cystoadenoma of the Body of the Pancreas

Introduction  

Annular pancreas (AP) is a rare anomaly due to malrotation of the pancreatic ventral bud during embryologic development. AP has been extensively described in the pediatric population; however, in adults, the incidence has been reported to be only 1 in 22,000 patients with only a few cases presenting with simultaneous mucinous cystadenoma described in the recent literature.     

Primary malignant fibrous histiocytoma of spleen with spontaneous rupture: a case report and literature review

Malignant fibrous histiocytoma (MFH) is the most common soft tissue sarcoma that mainly occurs in the lower and upper extremities, trunk and retroperitoneum. However, primary MFH of the spleen is especially rare. Only 11 cases of splenic MFH have been reported in the English literature. In this report, we describe a 35-year-old man who was found to have a large accumulation of free fluid in the abdominal cavity and a tumor mass 6 cm in diameter with rupture within the spleen by the abdominal ultrasonography. A splenectomy was performed and the histological diagnosis was malignant fibrous histiocytoma. The patient died 7 months after the operation as a result of generalized metastasis. Compared with the 11 previously documented patients of splenic MFH, our patient is the youngest and the first case with spontaneous rupture, which makes our case exceedingly rare. A literature review of primary MFH of spleen is also provided.     

胰腺浆液性囊腺瘤的CT表现

目的探讨胰腺浆液性囊腺瘤的CT表现。方法回顾性分析经手术病理证实的5例胰腺浆液性囊腺瘤的CT资料。结果肿瘤最长径2.5~11.9cm,平均6.5cm,边缘规整,分叶明显。CT平扫呈密度欠均匀的低密度肿物,CT增强扫描肿瘤内部的分隔中度或明显强化而呈网格状或蜂窝状;分隔厚度1.5~2.5mm,平均2.1mm;肿瘤内部囊的数目较多,直径1.6~12mm。1例肿瘤中心有粗大的点状钙化。结论大部分胰腺浆液性囊腺瘤的CT表现比较典型,结合临床特征并注意与粘液性囊性瘤或癌等鉴别可在术前作出正确诊断。     

A case of pancreatic serous cystadenoma obstructing the distal pancreatic duct.

We present a case of resected serous cystadenoma of the pancreas inducing marked dilatation of the main distal pancreatic duct. A 68-year-old woman, previously diagnosed with chronic pancreatitis, presented with upper abdominal pain. Abdominal US revealed a highly echoic mass in the pancreas. A CT scan disclosed a low density mass in the pancreas and dilatation of the main peripheral pancreatic duct. The mass demonstrated homogeneous and high signal intensity on T2-weighted magnetic resonance imaging (MRI). Selective abdominal arteriography showed the mass strained by the celiac artery. The tumor markers were CEA (2.4 ng/ml) and CA19-9 (6.1 U/ml). After the diagnosis of serous cystadenoma of the pancreas, the patient underwent distal pancreatectomy and splenectomy. The tumor (2.5 cm in diameter) consisted of grayish-white nodules and occupied the body of the pancreas. The tail of the pancreas was atrophic. Histopathological examination of the specimen showed a multilocular lesion containing numerous cysts with the inner surfaces evenly lined by one layer of cuboid or flat epithelial cells which stained positive for periodic acid-Schiff (PAS), evidencing serous cystadenoma. The patient is doing quite well one and a half years after the operation.     

Primary malignant fibrous histiocytoma of the lung. A clinicopathologic and ultrastructural study of five cases

Primary sarcomas of the lung are rare malignant neoplasms which have usually been classified as fibrosarcomas or as leiomyosarcomas. Only five cases of primary malignant fibrous histiocytoma (MFH) of the lung have previously been reported in the literature. The authors reviewed 10 cases of primary pulmonary sarcoma of the lung seen at their institution during the last 12 years, and five of these cases met the morphologic criteria for the diagnosis of MFH. The morphology in these cases is identical to MFH of other body locations, and shows a combination of fibroblast-like and histiocyte-like cells at both the light and electron microscopic level. By electron microscopic examination there was no evidence for anaplastic carcinoma, rhabdomyosarcoma, or leiomyosarcoma. Of the three patients with adequate follow-up, one patient is alive without evidence of disease at 10 years, one died at 5 years without evidence of recurrent sarcoma, and one died at 7 months with liver metastasis. Two other patients are alive without recurrence at 8 months and 1 year, respectively, following surgery. Because MFH is primarily a tumor of the soft tissues of the extremities, patients with MFH of the lung must be carefully evaluated to rule out a metastatic origin. The reported prognosis in patients with primary MFH of the lung is poor, but early surgical resection does offer the possibility of a cure.     

A serous cystadenoma of the ovary of borderline malignancy with a fifteen-year history. A case report

A case of a serous cystadenoma of a ovary of borderline malignancy is reported. Sixteen years earlier, the patient had undergone an exploratory laparotomy because of ovarian tumor, and the histologic diagnosis had been a serous cystadenocarcinoma. Postoperative chemotherapy was not effective and drainage of the tumor fluid had been performed for 15 years, with the estimated drainage volume estimated to have reached, 1,000 1. Gradual malnutrition and marked tumor growth then become apparent. A reevaluation of the initial histologic slides and her clinical course strongly suggested a serous cystadenoma of borderline malignancy. Thus a tumor resection, a bilateral salpingo-oophorectomy, and a hysterectomy was performed. A histologic diagnosis of a resected specimen confirmed a serous cystadenoma of borderline malignancy and the histologic features were quite similar to those of the initial biopsy specimens. The patient is living well postoperatively for 8 months without postoperative chemotherapy.     

导致生长发育障碍的胰腺实性假乳头状瘤7例分析

目的分析胰腺实性假乳头状瘤（SPTP）同时合并生长发育障碍患儿临床特点以及误诊原因。方法对7例经影像学及手术切除肿瘤病理证实胰腺实性假乳头状瘤患儿的临床特点进行回顾性分析。结果7例患者（男性1例,女性6例）的腹部肿物直径平均为7．4±1．9cm（3．0～12．8cm）。7例患儿术前均被发现身高偏矮,平均身高SDS为-1．9±0．2（-2．7～-1．3）,其中5例生长速度仅为1．4±0．1cm／年。术后3个月患儿体重平均增加1．1kg,生长速度为4．4±0．1cm／年（n=6）,6个月为7．4±0．3cm／年（n=5,P〈0．05）,平均身高SDS为-1．0±0．3（-1．1～-0．4）。7例术前疑为胰腺良性肿瘤或胰腺神经内分泌肿瘤,其中2例误诊为无功能性胰岛细胞瘤,误诊为黏液性囊腺瘤、浆液性腺瘤、胰腺癌以及假性囊肿各l例。另有2例在外院行运动试验呈阴性,遂诊为人生长激素缺乏性矮小症,但我院行两项人生长激素（hGH）兴奋试验其hGH峰值均高于10ng／ml。1例女性SPTP患儿在外院磁共振检查发现垂体增大,误诊为垂体瘤。结论SPTP是非常罕见的低度恶性腹部肿瘤,多发于青少年,应注意与无功能性胰岛细胞瘤、黏液性囊腺瘤、浆液性腺瘤、胰腺癌以及假性囊肿的鉴别。该肿瘤可明显影响患儿的生长发育,手术切除肿瘤后生长发育延迟可以得到逆转。     

Giant mucinous cystic neoplasms of pancreas and liver with unusual adipose tissue component: a case report

Simultaneous occurrence of pancreatic and hepatic mucinous cystic neoplasms is very rarely reported in the literature. We present a case with extensive fatty component of the pancreatic tumour arising from the head of the pancreas and attaining a huge size before being treated by Whipple’s pancreatoduodenectomy and subsequently by a right hepatectomy for the hepatic tumour.Key Words: Pancreas, mucinous cystic neoplasm, giant, cystadenoma, whipple’s pancreatoduodenectomy, liver, hepatectomy, adipose     

A case of malignant ovarian tumor complicated by pregnancy, from the standpoint of tumor marker and quality of life

It has been reported that malignant ovarian tumor is complicated in a ratio of one to 25,000 pregnancies. In the present study, a 22-year-old, nulliparous woman was presented, who had had her right ovary extirpated for serous cystadenoma (low potential for malignancy) and had undergone chemotherapy previously. Seven years later, she was subjected to celiotomy in the fourth month of gestational age because of left ovarian tumor. Intraoperative work-up disclosed a mucinous cystadenoma (low potential for malignancy) of stage Ia (FIGO). Left salpingo-oophorectomy and omentectomy were undertaken, and fetus-bearing uterus was preserved for the sake of the patient. Serum CA 19-9, with a preoperative level of 106.7 U/ml, fell exponentially into the normal range one month after the operation, and she successfully gave birth to a baby at term. After that, combination chemotherapy was instituted against an elevated level of CA 19-9, and she has been in remission while caring for her child.     

Stromal sarcoma with features of giant cell malignant fibrous histiocytoma

We report a case of primary giant cell malignant fibrous histiocytoma (GCMFH) of the breast. A 56-year-old Japanese woman presented with a firm mass in the right breast. Mammography and ultrasonography revealed a well-circumscribed and lobulated mass in the upper outer quadrant of the right breast, indicative of a benign breast tumor or mucinous carcinoma. Magnetic resonance imaging revealed a restricted breast tumor without intraductal spread. Computed tomography and bone scintigraphy found no sites of distant metastases. Fine needle aspiration biopsy showed several clusters of atypical cells associated with numerous multinucleated giant cells. Breast-conserving surgery with axillary lymph nodes dissection was performed. Histological examination showed primary GCMFH of the breast. No metastases were identified in any of the 15 left axillary lymph nodes resected and surgical margins were free from tumor cells. The tumor was negative for both estrogen and progesterone receptor. Neither adjuvant chemoendocrine therapy nor postoperative radiotherapy was given, and the patient has remained disease free for 30 months postoperatively. To our knowledge, only 30 cases of primary MFH of the breast have been reported in the literature.     

An autopsied case of malignant fibrous histiocytoma of the spleen

An auspsied case of malignant fibrous histiocytoma (MFH) is reported. The patient, a 53-year-old male had developed splenomegaly 1 year before admission. On admission, hepatosplenomegaly and ascites were seen, with prominent leucocytosis in the peripheral blood, and the patient died of cachexia. On autopsy, the primary neoplasma was found in the spleen, with metastasis to the liver, vertebrae, lymph nodes and peritoneum. Histologically, to tumor showed a storiform arrangement of tumor cells, with foamy or hemosiderin-faden cytoplasm in places, corresponding to MFH. As far as we know, there have been no reports of primary MFH of the spleen in Japan.     

Myxoid malignant fibrous histiocytoma of the bladder

Although the most common soft tissue sarcoma of adulthood, malignant fibrous histiocytoma (MFH) is an extremely rare tumor of the urinary bladder. Only three well-documented cases have been reported in the world literature. The patient presented in this report represents the first case of the myxoid variant to develop in the urinary bladder. Whereas all previous patients with MFH of the bladder had intermittent hematuria, this patient's chief complaint was bladder outlet obstruction due to extension of the tumor into the prostate. He was managed with radical cystoprostatectomy, postoperative radiation therapy to the tumor bed, and adjuvant chemotherapy using doxorubicin. The patient tolerated the therapy well and was disease-free at the 3-year follow-up visit. The histogenesis, clinical features, pathologic characteristics, and treatment considerations of this rare bladder tumor are discussed in detail.     

A malignant fibrous histiocytoma of the breast

We have experienced treating a 49-year-old female with a malignant fibrous histiocytoma (MFH) arising from the right mammary gland. Nine months after surgery, lung metastasis occurred but the lung lesion proved to be resectable. Our findings suggested that the primary lesion had originated from a phyllodes tumor. MFH arising from the mammary gland is an extremely rare tumor, and only 12 such cases have been reported including that of our patient.     

Mucinous Adenocarcinoma of the Thymus: A Case Report

A mucinous adenocarcinoma of the thymus is rare, and there is presently limited information on the clinical aspects of this uncommon type of tumor. Only six cases have been reported in the literature. A 49-year-old Japanese male presented with an anterior mediastinal mass, and underwent a tumor resection. Histologically the tumor consisted of a mucinous adenocarcinoma with pleural dissemination. Postoperative radiotherapy and chemotherapy were performed. The patient is still alive with pleural dissemination 11 months after surgery.     

阑尾黏液性肿瘤的诊断和外科治疗

目的探讨阑尾黏液性肿瘤的诊断和外科治疗方式。 方法对中国医科大学附属第四医院2007年1月至2017年12月经手术治疗的阑尾黏液性肿瘤24例患者临床资料进行回顾性分析。 结果24例患者临床表现主要为阑尾炎和腹部包块,均经手术治疗,经病理确诊为阑尾黏液性囊腺瘤2例,低级别阑尾黏液性肿瘤12例,阑尾黏液腺癌10例。行单纯阑尾切除术5例,行右半结肠切除术15例,行回盲部切除术4例。24例均进行随访,2例发生腹腔广泛转移分别死于术后第2年、第3年,3例死于其他疾病,1例患者在术后1年复发,行2次手术后至今未见复发,随访其他患者未见复发与转移。 结论阑尾黏液性肿瘤临床罕见,缺乏特异性临床表现,术前诊断困难且误诊率高。阑尾黏液性肿瘤主要治疗方式为阑尾切除术、右半结肠切除术、回盲部切除术。