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Pancreatic cytology can make a real difference to the management of patients. However it is a challenge in those cases where a definitive diagnosis of malignancy cannot be made with confidence. This creates the need for a unified terminology and nomenclature system that provides intra- and interdepartmental guidance for diagnosis. The Papanicolaou Society of Cytopathology (PSC) has published new guidelines for pancreaticobiliary cytology, addressing indications, techniques, terminology and nomenclature, ancillary studies, and postprocedure management., (© 2016 John Wiley & Sons Ltd.)

Background: Lymphangioma of the pancreas is an extremely rare benign tumour of lymphatic origin, with only four cases diagnosed by EUS-FNA reported to date., Methods and Materials: Five cases of either cytologically or histologically diagnosed pancreatic lymphangioma with pre-operative cytological analysis by EUS-FNAC were reviewed., Results: All patients were female, with a mean age of 56.4 years. By imaging, the cystic lesions ranged in size from 2 to 7 cm (mean 4.5 cm) and were mainly located in the head of the pancreas. All cysts had thin walls and no cyst demonstrated a mural nodule. Diagnosis based on imaging features was benign in all cases due to the absence of high-risk features. Four samples were sent for biochemical analysis, which showed low CEA levels (range, <0.5-19.4 ng/ml; mean, 5.45 ng/ml) and CA 19.9 and CA 72.4 levels within normal range. All cyst fluids showed numerous small lymphocytes with no atypia; no epithelial cells were present, including no gastrointestinal contamination. Flow cytometry in two cases showed T lymphocytes with a mature phenotype. Surgical resection in two patients confirmed the cytological diagnosis. Benign clinical follow-up was available in three patients at 2, 3 and 3.5 years., Conclusion: A multimodal approach to cytological diagnosis (combining clinical, radiological and cyst fluid gross, biochemical and cytological characteristics) can lead to the diagnosis of this cystic neoplasm and distinguish it from other more common cysts in the pancreas, potentially avoiding the need for unnecessary surgery., (© 2011 John Wiley & Sons Ltd.)

Aims: To describe the histopathological features of heterotopic cutaneous meningeal tissue., Methods and Results: Nineteen cases were collected between 1993 and 2010. Immunohistochemistry for epithelial membrane antigen (EMA), neuron specific enolase (NSE), S100, glial fibrillary acid protein (GFAP), progesterone receptor (PR), CD31, glucose transporter-1 (Glut-1), podoplanin and NKI-C3 was performed. Lesions were congenital (100%) and presented as aplasia cutis with alopecia (63%) or lumps (37%), on the scalp (18 of 19) and sacral region. Resonance magnetic imaging revealed four underlying anomalies of the neuraxis. Histopathological analysis showed meningeal tissue arranged in four variably associated architectural patterns: fibrous (100%), pseudovascular (100%), cellular (68%) and pseudomyxoid (32%). Other features included collagen bodies (58%), calcifications (26%) and dermal melanocytes (32%). Heterotopic brain tissue or heterotopic ependymal cyst was associated in two cases. Arachnoidal cells expressed EMA and NSE, but not S100 protein, CD31 or GFAP. They expressed podoplanin (93%), especially in pseudovascular areas, NKI-C3 (79%), and less frequently Glut-1 (46%) and PR (30%)., Conclusions: Histopathological features of cutaneous meningeal heterotopias are various and sometimes misleading. Fibrous lesions should not be misdiagnosed as aplasia cutis. Podoplanin-positive pseudovascular spaces represent the main pitfall and should not be diagnosed as lymphangioma. Correct diagnosis is confirmed by EMA and NSE coexpression within the lesion., (© 2011 Blackwell Publishing Limited.)

This review evaluates changes in vascular nomenclature particularly in the category of vascular tumours of intermediate malignancy that includes the various haemangioendotheliomas, Kaposi's sarcoma and giant cell angioblastoma. Recently described entities in this category, including the latter tumour and composite haemangioendothelioma, are described. Several newly characterized vascular tumours not included in recent classifications of vascular tumours are also summarized. These include acquired elastotic haemangioma, symplastic haemangioma, cutaneous epithelioid angiomatous nodule and cutaneovisceral angiomatosis with thrombocytopenia. The newly introduced clinical classification of congenital haemangiomas into non-involuting and rapidly involuting variants and radiation-induced atypical vascular lesions are also reviewed.

Aims: The aim of this report is to summarize the clinicopathological findings of five cases of a pseudosarcomatous vascular proliferation of the skin at the site of radiotherapy following surgery for carcinoma of the breast and carcinoma of the endometrium. To our knowledge, only five cases of this presumably rare pseudomalignant proliferation have been reported previously., Methods and Results: All patients were females ranging in age from 44 to 70 years. The lesions appeared as solitary or multiple papules or vesicles localized to the field of radiation. Microscopically, they were composed of vascular spaces that exhibited atypical features without qualifying for a diagnosis of angiosarcoma., Conclusions: Our cases provide additional evidence supporting the benign nature of this atypical vascular proliferation, not recurring, never developing metastases and being cured readily by local excision. Clinical, histopathological and ultrastructural findings suggest a lymphatic origin. Whether these lesions represent a neoplastic or a reactive condition secondary to radiotherapy is unclear. The name 'benign lymphangiomatous papules of the skin following radiotherapy' is proposed.

Lymphangioma of the oesophagus is an extremely rare entity, with only nine cases having been reported worldwide. We report on a 52-year-old woman with oesophageal lymphangioma, diagnosed using endoscopic ultrasonography and endoscopically resected. No case of malignant transformation of the lymphangioma has been reported in the literature. Endoscopic resection seems to be a minimally invasive method that is appropriate both for the removal of the tumour and precise diagnosis.

This review summarizes the clinicopathological features of a number of vascular tumours that have been characterized only in recent years. These include: glomeruloid haemangioma, a multifocal vascular lesion associated with POEMS syndrome: Kaposi-like infantile haemangioendothelioma, a borderline malignant tumour occurring in the retroperitoneum of infants, mimicking Kaposi's sarcoma histologically; giant cell angioblastoma, characterized by proliferated vessels with a granuloma-like appearance; benign lymphangioendothelioma (progressive lymphangioma), slowly-growing macule or plaque over the trunk or limb, mimicking low-grade angiosarcoma histologically; targetoid haemosiderotic haemangioma, a benign lesion with a distinctive annular appearance and histologically overlapping with benign lymphangioendothelioma; spindle cell haemangioendothelioma, a lesion located mostly in the distal extremities, characterized by cavernous vascular spaces, spindle cells with interspersed narrow vascular channels and scattered plump vacuolated endothelial cells; acquired tufted angioma, characterized by 'cannon-ball' involvement of the dermis by lobules of pericyte-rich capillaries; sinusoidal haemangioma, a distinctive variant of cavernous haemangioma which may be confused with angiosarcoma; and epithelioid angiosarcoma, a highly aggressive tumour of deep soft tissue mimicking metastatic carcinoma and co-expressing endothelial and epithelial markers.

We present a 27-year-old woman with non-systemic diffuse lymphangiomatosis of spleen and liver. The tumour consisted of capillary and cavernous lymphatic vessels located in abundant fibrous tissue. The vascular endothelium showed immunoreactivity for factor VIII-related antigen. A basal lamina could be demonstrated immunohistochemically and by electronmicroscopy.

We report a case of heterotopic brain which presented as a cystic mass in the palate and which clinically was thought to be a cystic hygroma. Histologically, there was a remarkable proliferation of choroid plexus-like structures which we believe to have been responsible for the production of cerebrospinal fluid. We believe heterotopic brain to result from early displacement of multipotential cells and that the presence of cerebrospinal fluid within extracranial brain tissue does not imply an intracranial communication.

A total of 147 specimens from 93 patients with penile lesions were examined at Nagasaki University Hospital during a 27-year period from 1961 to 1987. The most frequent malignant tumor was squamous cell carcinoma (SCC, 33 cases, 35.5%), followed by extramammary Paget's disease, transitional cell carcinoma, and Bowen's disease. The benign tumors and tumor-like lesions included condyloma acuminatum, cyst of the genitoperineal raphe, and lymphangioma. SCC of the penis accounted for less than 0.1% of all specimens and less than 0.62% of malignant tumors in men filed at our hospital. True phimosis accompanied 81.5% of the SCC cases. The 5- and 10-year survival rates for SCC were 77.2% and 71.3%, respectively. Two patients died of penile SCC. It was considered that an absence of both keratohyaline granules in the granular layer and melanin pigment in the basal layer can serve as a useful histologic indicator for diagnosis of well differentiated SCCs that are otherwise difficult to identify.

A case of lymphangioma of the pancreas in a 49-year-old woman is presented. The patient noticed a left hypochondral mass, which was clinically diagnosed as a multicystic tumor of the pancreas. A partial pancreatectomy and splenectomy were performed. The surgical specimen showed a multicystic mass measuring 13 X 9 X 5 cm which occupied the anterior region of the body and tail of the pancreas. From the light and electron microscopic findings, the multicystic mass was diagnosed as a lymphangioma with cystic and cavernous features. A small glucagonoma was incidentally found in the tail of the resected pancreas. The previous literature of pancreatic lymphangiomas is reviewed and the clinico-pathologic characteristics of this tumor are discussed.

We are reporting a rare case of an adenomatoid tumor of the uterus having multicystic gross appearance. A 32-year-old woman complaining of dysmenorrhea had multicystic mass lesions on the posterior wall of the right cornual region of the uterus. The specimen showed a honeycomb appearance with mucoid content. Microscopically, numerous gland-like spaces lined with low cuboidal cells were observed beneath the serosa, and mucopolysaccharide material accumulated in the cystic spaces forming, honeycomb-like lesions surrounded by myometrial tissue. The postoperative course was uneventful and the patient was discharged on the 9th day after the operation. "Cystic adenomatoid tumor of the uterus" was diagnosed. The patient is being followed up continuously, so far without recurrence.

Lymphangiomas of the pancreas are rare. Only six cases have been reported. An additional case of lymphangioma of the pancreas in a 20 year old male which was demonstrated by computerized tomography and treated with complete surgical excision is reported.

A 34-year-old black South African male was treated for lymphangioma-like clinical Kaposi's sarcoma. The disease followed a rapidly fatal course and was characterized by unexplained recurrent pleural effusions and severe refractory anaemia due to the presence of dilated vascular channels in the bone marrow. Definitive diagnosis was only made at autopsy. Lymphangiomatous changes in the skin without spindle cell formation caused diagnostic difficulties.

The present study consists of 651 cases of soft tissue tumors originating in the head and neck area. Among these tumors 628 were benign (96%), while there were only 23 malignant cases (4%). In the benign group hemangiomas showed the highest incidence (47%) followed by tumors of adipose tissue (19%) and those of peripheral nerves (12%). Rare tumors consisted of nodular fascitis, hibernoma, lipoblastomatosis, glomus tumor, granular cell tumor, and chondroma. The majority of cases in the malignant group were either those of muscle origin or blood vessel origin. Excepting for two cases of rhabdomyosarcoma of the embryonal type and two cases of angiosarcoma, all showed recurrence and/or metastasis and terminated in death. Three cases of liposarcoma of either well-differentiated or myxoid type had a good prognosis with all three living for 3 or more years without any recurrence. Other malignant tumors consisted of malignant schwannoma, fibrosarcoma, malignant fibrous histiocytoma, alveolar soft part sarcoma, and chordoma. The age, sex, anatomical location, and differential diagnosis have also been described.