Transhiatal and transthoracic resection in adenocarcinoma of the esophagus: Does the operative approach have an influence on the long-term prognosis?

Background

Goblet cell carcinoid (GCC) of the appendix is a rare neoplasm that share histological features of both adenocarcinoma and carcinoid tumor. While its malignant potential remains unclear, GCC's are more aggressive than conventional carcinoid. The clinical presentations of this neoplasm are also varied. This review summarizes the published literature on GCC of the appendix. The focus is on its diagnosis, histopathological aspects, clinical manifestations, and management.

Methods

Published studies in the English language between 1966 to 2004 were identified through Medline keyword search utilizing terms "goblet cell carcinoid," "adenocarcinoid", "mucinous carcinoid" and "crypt cell carcinoma" of the appendix.

Results

Based on the review of 57 published papers encompassing nearly 600 diagnosed patients, the mean age of presentation for GCC of the appendix was 58.89 years with equal representation in both males and females. Accurate diagnosis of this neoplasm requires astute observations within an acutely inflamed appendix as this neoplasm has a prominent pattern of submucosal growth and usually lacks the formation of a well-defined tumor mass. The mesoappendix was involved in 21.64% followed by perineural involvement in 2.06%. The most common clinical presentations in order of frequency were acute appendicitis in 22.5%; asymptomatic in 5.4%; non-localized abdominal pain in 5.15% and an appendicular mass in 3.09%. The most common surgical treatment of choice was appendectomy with right hemicolectomy in 34.70% followed by simple appendectomy in 24.57%. Concomitant distant metastasis at diagnosis was present in 11.16% of patients with the ovaries being the most common site in 3.60% followed by disseminated abdominal carcinomatosis in 1.03%. Local lymph node involvement was seen in 8.76% of patients at the time of diagnosis. The reported 5-year survival ranges from 60 % to 84%. GCC's of the appendix remains a neoplasm of unpredictable biological behavior and thus warrants lifelong surveillance for recurrence of the disease upon diagnosis and successful surgical extirpation.

Conclusion

GCC of the appendix is a rare neoplasm. Due to its wide range of presentation, this tumor should be considered as a possible diagnosis in many varied situations leading to abdominal surgery. Histopathological features such as increased number of Paneth cells, increased amount of mucin secretion and presence of pancreatic polypeptide may predict a more aggressive behavior. The advocated plan of management recommended for patients with tumors that involve the adjacent caecum or with high-grade tumors with histological features such as an increased mitotic rate involve initial appendectomy with completion right hemicolectomy due to the high possibility of local recurrence with intraperitoneal seeding prior to lymph node involvement and a 20% risk of metastatic behavior. In female patients with GCC of the appendix regardless of age, bilateral salpingo-oophorectomy is advocated. In cases with obvious spread of the disease chemotherapy, mostly with 5-FU and leucovorin is advised. Cytoreductive surgery with adjuvant intraperitoneal chemotherapy can offer improved survival in cases with advanced peritoneal dissemination.     

Neuroendocrine cells along the digestive tract express neuropilin-2

Neuropilin-2 (np-2) is a receptor for semaphorin-3F (sema-3F) and semaphorin-3C (sema-3C). These semaphorins repel tips of growing axons that express np-2. In addition, np-2 functions as a receptor for heparin binding forms of the angiogenic factor vascular endothelial growth factor (VEGF) such as VEGF145 and VEGF165. We report that np-2 is strongly expressed in neuroendocrine cells located all along the human digestive tract. Confocal fluorescent microscopy revealed that np-2 is concentrated in vesicle-like structures located near the nucleus at the basolateral side of these cells. In the colon, the np-2-expressing subpopulation of neuroendocrine cell is almost identical with the serotonin-producing subpopulation of neuroendocrine cells. Gastrointestinal carcinoid tumors are digestive tract tumors that develop from neuroendocrine cells. Interestingly, most of the carcinoid tumors derived from the colon and the appendix did not contain np-2-producing cells. However, some carcinoid tumors derived from the small intestine and stomach did express low levels of np-2 in isolated foci of cells. By contrast, strong serotonin and chromogranin-A expression was observed in all of the carcinoid tumors that were examined. These results suggest that loss of np-2 expression may accompany tumor progression in carcinoid tumors.     

Primary pulmonary malignant peripheral nerve sheath tumor: a case report

BACKGROUND: Although the histologic features of malignant peripheral nerve sheath tumor (MPNST) have been described, the cytologic features of primary pulmonary MPNST have not been reported in the literature. CASE: We report a case of primary pulmonary MPNST in a 78-year-old man. Follow-up computed tomography of colon cancer, renal cancer, penile cancer and gingival cancer revealed a nodular lesion, 12 mm in diameter, in the right upper lobe of the lung. In frozen section, a diagnosis of malignant neoplasm, not otherwise specified, was rendered for the imprinting specimen and histologic specimen. Imprinting specimens were composed of small cellular aggregates and discohesive neoplastic cells with obvious malignant features. Histologically, spindle cells with pleomorphic nuclei arranged infascicular patterns and multinucleated tumor giant cells were also observed. More than 25 mitotic figures were observed per 10 high-power fields. Tumor cells were positive only for vimentin and S-100, and the Ki-67 labeling index was 10%. Clinical and imaging investigation failed to identify an alternative primary site. We histologically diagnosed this case as primary pulmonary MPNST. CONCLUSION: MPNST has a varied cytomorphology with frank nuclear atypia showing no definite differentiation. Multinucleated neoplastic giant cells with immunopositivity for S-100 may permit more accurate diagnosis of MPNST.     

Myeloid sarcoma and adenocarcinoma of the large bowel as collision tumors: a case report

Myeloid sarcoma is a rare tumor composed of myeloid cells, localized in an extramedullary site, which may be associated with a concurrent myeloid neoplasm involving the bone marrow, although such an association is not required. Most patients present with acute myeloid leukemia and their prognosis is poor. We describe the case of a 76-year old woman with an adenocarcinoma of the right colon infiltrating the subserosa synchronous with a myeloid sarcoma at the same site; one pericolic lymph node was infiltrated by both tumors. The peculiarities of this case are the clinical presentation (as an acute abdomen due to subserosa infiltration by the myeloid sarcoma), the coexistence of a myeloid sarcoma with an adenocarcinoma of the right colon, and the absence of progression to acute leukemia. Coexistence of myeloid sarcoma and adenocarcinoma in the colon is probably incidental, and so it appears likely that the two different tumours arose from different mechanisms. However, a possible common background is conceivable. Some authors have found that p53 has a pivotal role in driving the maturation of myeloid stem cells and p53 is, also, involved in colon carcinogenesis. In our case, it may be hypothesized that synchronous heterogeneous mutations occurred in different types of committed cells or in stem cells secondary to p53 loss. Since only one case report has evaluated the correlation between myeloid sarcoma and adenocarcinoma of the large bowel, further immunohistochemical and molecular studies are needed to clarify the pathogenetic relationship between them.     

Tension pneumocephalus secondary to osteoradionecrosis of the clivus

We report the case of a 36 year-old man with relapsing undifferentiated nasopharyngeal carcinoma treated with a re-irradiation Cyberknife, who subsequently developed tension pneumocephalus due to a cerebrospinal fluid leakage located at the clivus. The fistula was caused by osteonecrosis of the skull base secondary to the tumor invasion and to the sequelae of the radiotherapy. An endoscopic endonasal technique was used in order to repair the defect, with a peduncolated nasoseptal flap harvested to perform the skull base reconstruction. In this paper, we discuss the importance of identifying this possible complication related to radiotherapy in the management of neoplasm along the skull base; moreover, the role of endoscopy in the diagnosis and treatment of skull base fistulas is also described and commented.     

Osteoclastomalike giant cell tumor of the parotid gland: report of a case with fine needle aspiration diagnosis

BACKGROUND: Osteoclastomalike giant cell tumor of the parotid gland has been reported rarely. The tumor has occurred rarely at many sites, such as thyroid, pancreas, soft tissue, breast, skin, heart, colon, lung, kidney, ovary and bladder. The exact origin of the tumor is unclear. However, osteoclastlike giant cells have been considered either part of a stromal process reactive to a neoplasm or a component of a primary neoplasm. CASE: A 35-year-old female presented with a mass in the left parotid gland clinically diagnosed as a pleomorphic adenoma. Fine needle aspiration (FNA) was advised before surgical excision. FNA smears revealed numerous osteoclastlike, multinucleated giant cells and many malignant-looking mononuclear cells. The smears were diagnosed as positive for malignancy, suggestive of osteoclastomalike giant cell tumor. The tumor was excised, and histopathologic study confirmed the cytologic diagnosis. CONCLUSION: The cytologic findings of osteoclastomalike giant cell tumor of the parotid gland have not been previously reported. FNA aided the diagnosis and planning of treatment. FNA is important in the diagnosis of parotid tumors.     

Primary effusion lymphoma. A case report

BACKGROUND: Primary effusion lymphoma (PEL) is a rare type of lymphoma that presents as an effusion, seldom with evidence of a solid neoplasm elsewhere; thus, cytology is the basic diagnostic method. It usually occurs in HIV-positive males with a history of Kaposi's sarcoma (KS), and DNA sequences of human herpesvirus 8 (HHV-8) are detected by molecular analysis. The distinct morphologic, immunophenotypic, molecular and clinical characteristics render this neoplasm a new pathologic entity. CASE: A 57-year-old, HIV-positive man presented to the hospital with ascites and absence of neoplasm on radiologic investigation. Cytologic evaluation of the ascitic fluid revealed the presence of highly atypical, pleomorphic lymphoid cells. Immunocytochemistry of the lymphoma cells was positive for CD45 (leukocyte common antigen), CD30 and epithelial membrane antigen antigens and negative for panB, panT and cytokeratin antigens. DNA sequences of HHV-8 were identified by polymerase chain reaction (PCR), and DNA ploidy analysis showed aneuploidy. The patient died 5 months after the diagnosis. CONCLUSION: Conventional and ThinPrep (Cytyc Corp., Boxborough, Massachusetts, U.S.A.) cytology, in combination with immunocytochemistry and PCR for HHV-8 DNA sequences, can lead to an accurate diagnosis of PEL. DNA ploidy analysis confirms the aggressive nature of this neoplasm.     

Effusion cytology of endodermal sinus tumor of the colon. Report of a case

A three-year-old boy presented with a peritoneal effusion due to an occult endodermal sinus tumor in the ascending colon. Cytologic examination of the ascitic fluid revealed clusters of round plump cells that had large hyperchromatic nuclei, finely stippled chromatin, multiple prominent nucleoli and vacuolated or mucin-containing cytoplasm, features suggesting an adenocarcinoma. The colonic primary was discovered during emergency laparotomy performed due to suspected acute hemoperitoneum. Endodermal sinus tumor should be included in the differential diagnosis when cytologic features reminiscent of adenocarcinoma are encountered in a fluid specimen from a child, especially if there is a history of a gonadal or extragonadal childhood neoplasm.     

Fine needle aspiration cytology of atypical carcinoid of the lung

The cytologic features of eight atypical carcinoid tumors of the lung, as observed in fine needle aspiration (FNA) specimens, are described in detail. They were compared with 21 pulmonary squamous-cell carcinomas, 16 adenocarcinomas, 5 small-cell undifferentiated carcinomas, 3 large-cell undifferentiated carcinomas and 1 typical carcinoid tumor. Atypical carcinoid tumor was easily distinguished from the other pulmonary neoplasms in most instances. Only two poorly differentiated squamous-cell carcinomas (one of which had atypical carcinoid as a component) and one small-cell undifferentiated carcinoma had similar cytologic features. One atypical carcinoid also had cytologic features similar to small-cell undifferentiated carcinoma. Because atypical carcinoid and small-cell undifferentiated carcinoma, at times, may be difficult to separate in FNA specimens, surgical resection of all stage I neoplasms with cytologic features evocative of either neoplasm is recommended.     

Oral desmoplastic melanoma mimicking inflammatory hyperplasia

doi: 10.1111/j.1741‐2358.2011.00482.x Oral desmoplastic melanoma mimicking inflammatory hyperplasia Introduction: Desmoplastic melanoma (DM) arising in the oral cavity is a rare neoplasm that may be confused with a variety of non‐melanocytic benign or malignant lesions. Objectives: To present a case of DM in the oral mucosa mimicking fibrous inflammatory hyperplasia, discusses the difficulties involved in the diagnosis and offers a literature review on the clinicopathologic and immunohistochemincal aspects of this neoplasm. Case report: A 62‐year‐old white male, smoker, was referred with a chief complaint of pain and swelling in the palate. The oral examination revealed multiple brown‐to‐black patches and a non‐pigmented sessile nodule located on the mucosa of the hard palate. The clinical diagnosis of the pigmented lesions was either oral melanosis or melanoma. The nodular lesion was clinically diagnosed as fibrous inflammatory hyperplasia. Incisional biopsy was performed on the pigmented lesion and the microscopic sections revealed a melanotic macule. The nodular lesions histologically revealed an amelanotic desmoplastic melanoma. Conclusions: Reactive lesions close to a pigmented area should be investigated with great care.     

Primary hepatic carcinoid tumor

Primary hepatic carcinoid tumor is rare and poses a challenge for diagnosis and management. We presented a case of primary hepatic carcinoid tumor in a 53-year-old female with a complaint of right upper abdominal pain. Computer tomography scans revealed a hypervascular mass in segment 4 of the liver. An ultrasonography-guided biopsy showed a carcinoid tumor. No other lesions were found by the radiological investigations. Surgery resection was performed and histopathological examination revealed a primary hepatic carcinoid tumor. Three years later, recurrence was found and transcatheter arterial chemoembolization was performed. After transcatheter arterial chemoembolization, the patient has been free of symptom and had no radiological disease progression for over 6 months. Surgical resection combination with transcatheter arterial chemoembolization is effective to offer excellent palliation.     

Fine needle aspiration biopsy of primary mucinous carcinoma of the skin: a case report

BACKGROUND: Primary mucinous carcinoma of the skin is a rare neoplasm of sweat gland origin. To date there are only 2 case reports in English describing its features on fine needle aspiration biopsy (FNAB). We describe an additional case and review the literature regarding this entity. To the best of our knowledge, this is the first reported case with a sentinel lymph node biopsy. CASE: A 78-year-old woman presented with a 3-cm left scalp mass at an outside institution. Following incomplete excision, multiple subcentimeter nodules developed in the skin adjacent to the biopsy site. FNAB of the nodules confirmed a recurrence of mucinous carcinoma. Clinical examination and extensive radiographic studies did not reveal primary disease elsewhere, thus supporting a diagnosis of primary mucinous carcinoma of the skin. At the time of wide excision of the residual tumor, sentinel lymph node biopsy revealed a single focus of micrometastasis. The patient declined adjuvant therapy and was disease free 6 months after the initial diagnosis. CONCLUSION: Cutaneous mucinous carcinoma is a tumor characterized by bland histocytologic features and abundant extracellular pools of mucin. Without a high index of suspicion, this rare entity may be overlooked or misdiagnosed. Numerous benign and malignant mucin-producing primary and secondary mimics exist, and immunohistochemistry offers limited benefits in differentiating them. Cytologic diagnosis of primary mucinous carcinoma of the skin is possible; however, correlation of clinical, radiologic and pathologic features is necessary to arrive at an accurate diagnosis.     

Tailgut cyst associated with a carcinoid tumor: case report and review of the literature

We report the case of a 49-year-old woman who presented a tailgut cyst lined by a variety of epithelium including squamous, columnar and transitional. Fortuitously a microscopic carcinoid tumor expressing immunohistochemically neuroendocrine markers was identified in the cystic wall. Tailgut cysts are congenital abnormalities located in the presacrococcygeal area occurring usually in adult patients. Clinical diagnosis is difficult because they are often asymptomatic. Patients may present symptoms resulting from local mass effects or complications. The differential diagnoses include rectal duplication cysts, cystic sacrococcygeal teratomas, epidermal cysts, epidermoid cysts, anal duct or gland cysts. Magnetic resonance imaging has recently become the modality of choice to image the cyst. Malignant transformation is rare; 23 cases including 10 carcinoid tumors have been reported in the literature. To our knowledge, this is the eleventh case of carcinoid tumor arising in a tailgut cyst.     

Pulmonary Atypical Carcinoid Tumor with Metastatic Involvement of the Pituitary Gland Causing Functional Hypopituitarism

ObjectiveTo report a unique case of a peripherally located pulmonary atypical carcinoid tumor with metastatic involvement of the pituitary, manifesting with evidence of functional hypopituitarism and compressive symptoms of dysarthria, bitemporal loss of vision, and ataxia.MethodsWe present a case report, including detailed laboratory, radiologic, and pathologic findings in a 50-year-old woman with a peripherally located pulmonary atypical carcinoid tumor and a lesion metastatic to the pituitary gland. The pertinent literature is also reviewed.ResultsA 50-year-old woman with a medical history of metastatic pulmonary atypical carcinoid tumor presented with symptoms of bitemporal hemianopia, ataxia, and dysarthria. Laboratory evaluation revealed functional hypopituitarism, and magnetic resonance imaging of the brain with use of gadolinium contrast demonstrated a pituitary lesion measuring 3.5 by 2.5 by 2.5 cm. Visual field testing revealed bitemporal superior quadrantanopia, consistent with a pituitary lesion of the chiasm. Computed tomographic angiography excluded the presence of an aneurysm of the internal carotid artery. Subsequently, the patient underwent subtotal transsphenoidal hypophysectomy. Findings on pathology examination were consistent with a lesion metastatic from the primary pulmonary atypical carcinoid tumor. The patient subsequently received monthly octreotide injections as well as chemotherapeutic regimens of capecitobine and temozolomide. At 18-month follow-up, disease had not recurred.ConclusionThis case of metastatic involvement of the pituitary gland from a peripherally located pulmonary atypical carcinoid tumor manifesting with evidence of functional hypopituitarism is highly uncommon. To our knowledge, such a case has not been previously reported. The presentation of an advanced pituitary metastatic lesion can be particularly dramatic, and the course can be potentially complicated. Therefore, heightened awareness of the possibility of a pituitary lesion metastatic from a pulmonary atypical carcinoid tumor is important. (Endocr Pract. 2007;13:291-295)     

Isolated carcinoid tumor metastatic to the thyroid gland: report of a case initially diagnosed by fine needle aspiration cytology

BACKGROUND: Neuroendocrine tumor metastatic to the thyroid gland is rare and may be difficult to differentiate from primary thyroid neuroendocrine tumors, such as medullary thyroid carcinoma (M/ITC). This report describes an unusual case of bronchial carcinoid metastatic to the thyroid diagnosed by fine needle aspiration cytology (FNAC). CASE: A 42-year-old woman with an undiagnosed bronchial carcinoid tumor presented to our clinic with a solitary nodule in the thyroid gland. FNAC of the nodule showed loosely cohesive groups of cuboidal tumor cells with scant, slightly granular cytoplasm; centrally located nuclei with a coarsely granular, salt-and-pepper chromatin pattern and inconspicuous nucleoli. Immunocytochemically the tumor cells were positive for neuron-specific enolase, chromogranin and synaptophysin and negative for thyroglobulin, calcitonin and carcinoembryonic antigen. The cytologic diagnosis of a metastatic neuroendocrine carcinoma was confirmed histologically. CONCLUSION: Metastasis to the thyroid gland may pose a diagnostic problem, particularly with tumors of neuroendocrine origin, as these have similar cytologic features in various organs. The correct preoperative cytologic diagnosis of metastatic carcinoid tumor in patients without a prior history of cancer and differential diagnosis with MTC are crucial because prognosis, workup and treatment are different in each.     

Stenosing colonic adenocarcinoma in a female rhesus monkey

Abstract: Laparoscopic surgery was performed on a 16 year-old female rhesus monkey presenting with chronic lethargy and inappetence. The procedure revealed a “napkin-ring” stricture located in the ascending large colon. Histologic evaluation of the colonic lesion exhibited large numbers of irregular acini lined by a single layer of well-differentiated neoplastic epithelial cells. Based on the gross and histopathologic findings a diagnosis of adenocarcinoma of the proximal colon was made.     

A cellular 57 kDa protein binds to two regions of the internal translation initiation site of foot-and-mouth disease virus

A ribosome-associated 57 kDa protein from rabbit reticulocytes was linked to the internal translation initiation site of foot-and-mouth disease virus by mild UV-irradiation. Binding studies with different RNA fragments revealed that this protein interacts with two distinct sites within the translational control region. One site is located approximately 400 nucleotides upstream from the translational start codon and the second binding site could be confined to 60 nucleotides preceding this codon. Both sequences coincide with hairpin structures at the two opposite ends of a secondary structure model of the internal ribosomal entry site proposed by Pilipenko et al. [(1989) Nucleic Acids Res. 17, 5701-5711].     

Cytologic findings in primary malignant carcinoid tumor of the cervix. Including immunohistochemistry and electron microscopy performed on cervical smears

The cytologic findings in a primary malignant carcinoid tumor of the cervix are presented. In addition to the presence in the smears of cells suggestive of squamous carcinoma and adenocarcinoma, which led to an initial diagnosis of adenosquamous carcinoma, there were multinucleated giant cells with prominent, reddish nucleoli, finely granular chromatin and grayish-blue to eosinophilic cytoplasm, as well as smaller pleomorphic cells, against a tumor-diathesis type of background. Immunocytochemistry performed on the cervical smears showed the presence of serotonin, and ultrastructural analysis revealed abundant intracytoplasmic, membrane-bound granules in malignant cells, thus confirming the diagnosis of a carcinoid tumor.     

Oncocytic carcinoid tumor of the lung: a case report of diagnostic pitfall in filter membrane preparation of bronchial washings

BACKGROUND: Oncocytic carcinoid tumor of the lung is a rare variant of pulmonary carcinoid. This report describes the morphologic appearance of this rare tumor on filter membrane preparation along with potential pitfalls. CASE: A 49-year-old woman presented with cough and expectoration. On chest radiograph a mass lesion was seen in the upper zone of the right lung. Bronchial washings were sent for evaluation. On filter membrane (Millipore) preparation of bronchial washing the possibility of a non-small cell carcinoma, possibly squamous, was suggested. Right upper lobectomy was subsequently performed and a histologic diagnosis of oncocytic carcinoid given. The cytomorphologic features of this tumor on the Millipore preparation were reviewed. CONCLUSION: Differential diagnosis of oncocytic carcinoid should be kept in mind while assessing cytologic material when tumor cells show abundant granular cytoplasm and prominent nucleoli. Oncocytic carcinoid also must be differentiated from oncocytoma and granular cell tumor. Immunocytochemistry and electron microscopy are useful in confirming the diagnosis.     

Tryptophan environments in glutathione transferase of human placenta from temperature-dependent phosphorescence studies

An investigation of the tryptophan emission properties of glutathione transferase from human placenta was conducted in order to characterize the environments of the two aromatic residues. The low-temperature phosphorescence spectra and temperature dependence of the phosphorescence quantum yield of the tryptophan residues revealed a difference in the chemical nature and dynamical structure of the surrounding protein matrix. Thus, one tryptophan residue seems to be deeply embedded within the polypeptide in a rigid weakly polar environment, characteristic of a beta-type secondary structure. The other is located in a more polar site, probably near the surface, in a rather flexible region of the macromolecule. At high temperature, the heterogeneity in the triplet lifetime of the internal residue attests to the presence of multiple conformers which are not in rapid equilibrium in the phosphorescence time scale. The anisotropy of the phosphorescence emission of glutathione transferase indicates that no energy transfer occurs between the two residues, and measurement of the rotational correlation time yields an hydrodynamic volume which is in good agreement with the molecular weight reported in the literature for the dimer.