ADENOID SQUAMOUS CELL CARCINOMA OF THE SKIN OVERLYING THE RIGHT BREAST: An Autopsy Case Clinically Manifested with Rapid Growth and Widely Spreading Metastases

An autopsy case of a 62-year-old woman with a poorly differentiated, aggressive form of adenoid squamous cell carcinoma arising in the skin overlying the right breast was studied. The tumor, 9×8cm in diameter, had rapidly enlarged since one year before admission from a verrucous lesion of 20 years duration. The histologic features of the tumor showed a well-differentiated squamous cell carcinoma mainly in the superficial areas, which transformed into, with a zone of transition in between, an alveolar or adenoid structure in the deep invading portion. The adenoid tumor cells exhibited an undifferentiated appearance with prominent nucleoli and frequent mitotic figures. These cells partly showed dyskeratotic or acantholytic features. Mucin was negative. The patient died at 8 months after the operation. Autopsy revealed widely spreading metastases in which an adenoid structure was outstanding. These unusual pathological features and an aggressive behavior of this tumor, which were hitherto rarely described for adenoid squamous cell carcinoma, seemed to be a poorly differentiated variant of the tumor. This malignant transformation might be derived from loss of cohesion of the pre-existing usual well-differentiated squamous cell carcinoma in the basal and parabasal layers, inparting marked invasiveness of these cells into the supporting connective tissue.     

Adenoid squamous cell carcinoma of the skin overlying the right breast. An autopsy case clinically manifested with rapid growth and widely spreading metastases

An autopsy case of a 62-year-old woman with a poorly differentiated, aggressive form of adenoid squamous cell carcinoma arising in the skin overlying the right breast was studied. The tumor, 9 X 8 cm in diameter, had rapidly enlarged since one year before admission from a verrucous lesion of 20 years duration. The histologic features of the tumor showed a well-differentiated squamous cell carcinoma mainly in the superficial areas, which transformed into, with a zone of transition in between, an alveolar or adenoid structure in the deep invading portion. The adenoid tumor cells exhibited an undifferentiated appearance with prominent nucleoli and frequent mitotic figures. These cells partly showed dyskeratotic or acantholytic features. Mucin was negative. The patient died at 8 months after the operation. Autopsy revealed widely spreading metastases in which an adenoid structure was outstanding. These unusual pathological features and an aggressive behavior of this tumor, which were hitherto rarely described for adenoid squamous cell carcinoma, seemed to be a poorly differentiated variant of the tumor. This malignant transformation might be derived from loss of cohesion of the pre-existing usual well-differentiated squamous cell carcinoma in the basal and parabasal layers, inparting marked invasiveness of these cells into the supporting connective tissue.     

Plasmablastic lymphoma of the retroperitoneum in an HIV‐negative patient

Herein is reported a case of plasmablastic lymphoma (PBL) of the retroperitoneum in an HIV‐negative patient. This is the first reported case of PBL at this location and of PBL from Japan in the English‐language literature. A 76‐year‐old Japanese man was admitted to hospital with a chief complaint of right inguinal lymph node swelling. Lymph node biopsy indicated large tumor cells with both diffuse and cohesive growth patterns, and conspicuous tumor cell proliferation in lymph node sinuses. The initial pathological diagnosis was metastatic carcinoma. The patient died approximately 1 month after admission, and autopsy showed that the main lesion was a very large retroperitoneal mass. On histology diffusely proliferated plasmablast‐like or immunoblast‐like tumor cells were identified, which were positive on immunohistochemistry for CD138 and negative for B‐cell and epithelial markers. Approximately 90% of the tumor cells were positive for Ki‐67. Tumor cells were diffusely positive for EBV‐encoded small RNA on in situ hybridization. The autopsy findings suggested a diagnosis of PBL. Accordingly, PBL should be considered as a differential diagnosis when lymph node biopsy findings resemble those of the present patient.     

Sacrococcygeal chordoma in infancy showing an aggressive clinical course: an autopsy case report

The autopsy case of a 3-year 6-month-old boy with chordoma arising in the sacrococcygeal region is presented. The primary lesion of the sacrococcygeal area was unresectable and lung metastasis was detected. He was treated with multi-agent systemic chemotherapy and radiation therapy, but the tumor was less responsive to these therapies. He died about one year after first admission. An autopsy revealed a massive sacrococcygeal mass and metastasis in the thoracic and lumbar vertebrae, retroperitoneal and mediastinal lymph nodes, and also in the bilateral lungs and liver. Histologically, the tumor was composed of 'pink' cells and scattered 'physaliphorous' cells with a myxoid matrix. Sacrococcygeal chordoma in infancy is very rare. Our case showed a highly aggressive clinical course.     

Xanthic variant of non-ossifying fibroma (so-called xanthofibroma) of the mandible. An ultrastructural study

An unusual case of a 37-year-old female with xanthomatous bone tumor of the right molar area of the mandible was presented. The tumor was asymptomatic and found to be a well-demarcated intraosseous radiolucent lesion on radiographic examination. Histologically the tumor consisted of two cell types, fibroblastic and xanthomatous cells. There was no osteoid, bone or cartilage formation. However, numerous psammomatous calcified bodies were seen in the fibrous area. Ultrastructural study showed fibroblastic cells in different stages of proliferation as the basis of the tumor which transform itself into xanthomatous cells. From the clinicopathologic findings, our case was thought to be a xanthic variant of non-ossifying fibroma (so-called xanthofibroma) of the mandible.     

XANTHIC VARIANT OF NON-OSSIFYING FIBROMA (SO-CALLED XANTHOFIBROMA) OF THE MANDIBLE

An unusual case of a 37-year-old female with xanthomatous bone tumor of the right molar area of the mandible was presented. The tumor was asymptomatic and found to be a well-demarcated intraosseous radiolucent lesion on radiographic examination. Histologically the tumor consisted of two cell types, fibroblastic and xanthomatous cells. There was no osteoid, bone or cartilage formation. However, numerous psammomatous calcified bodies were seen in the fibrous area. Ultrastructural study showed fibroblastic cells in different stages of proliferation as the basis of the tumor which transform itself into xanthomatous cells. From the clinicopathologic findings, our case was thought to be a xanthic variant of non-ossifying fibroma (so-called xanthofibroma) of the mandible.     

A case of unclassified multicystic biliary tumor with biliary adenofibroma features

A 40-year-old Japanese man was admitted to our hospital for evaluation of upper abdominal pain. Abdominal computed tomography (CT) revealed a well-circumscribed multicystic mass measuring approximately 7 × 6 cm. The mass contained a solid lesion measuring 3 × 2 cm. Biopsy of a swollen cervical lymph node led to a diagnosis of diffuse large B-cell lymphoma. After initial chemotherapy for lymphoma, the multicystic mass was surgically resected. The tumor was composed of a multicystic lesion and a solid lesion. Histopathologic examination of the multicystic lesion revealed that the locules were lined by biliary epithelium, demonstrating various degrees of cytological atypia. The stroma was fibrous, and the tumor showed marked apocrine snouts. Part of the tumor showed papillary growth with strong cytological atypia. The solid lesion showed tubulocystic proliferation of tumor cells, with prominent apocrine snouts, embedded in dense and partially hyalinized fibrous stroma. The morphology of the solid part was quite similar to that of reported biliary adenofibroma. Despite lengthy discussion, an appropriate pathological diagnosis could not be found among the current classifications of biliary tumor. The tumor was finally diagnosed as unclassified multicystic biliary tumor with adenofibroma features.     

Chondromyxoid fibroma of the distal phalanx of the great toe: A tumor with unusual histological findings

Chondromyxold fibroma (CMF) rarely arises in the distal phalanx of the foot and less than 20 cases have been reported In the literature. It has also been known to show a wide spectrum of histology mlmlcking other primary bone tumors. An unusual case of CMF arising in the dlstal phalanx of the left great toe is reported because of Its unique anatomic site of origin and histology. A 53-year-old female presented with a slow growing, painful great toe of the left foot which she had had for 3 years. She had first noticed the mass 25 years ago. On admission, plain X-ray revealed an osteolytic mass with a sclerotic margin expanding to the distal phalanx of the great toe. Interestingly, the lesion was microscopically composed of hypercellular chondromyxoid lobules separated by hypocellular fibrous tissue, which is in contrast to the typical histology of CMF. In addition, the lesion showed an aggregate of tumor cells with pleomorphic multinucleate or giant nuclel within the chondromyxold matrix, which were not similar to the osteoclast-llke type. Perhaps these unusual histologlcal findings may be associated with its long duration and presenting location.     

Tanycytic ependymoma: two case reports and review of the literature

Two cases of tanycytic ependymoma are reported, one in a woman aged 33 years and one in a man aged 36 years. The woman presented with lesions at L2 and L3 in the spinal cord and clinical symptoms suggested spinal cord compression, such as movement and sensory dysfunction. The man had a lesion located in the lateral ventricle and presented with headache, vomiting, and impaired vision. Both of these patients developed gradual increases in the intensity of their symptoms over a few months prior to admission. A spinal cord MRI of the woman showed a well-defined, solid cystic mass measuring 1.0 × 6.5 cm at L2 and L3 of the spinal cord. The mass showed low signal intensity on T2-weighted images in the intervertebral disc with equal signal intensity T1-/T2-weighted images. The signal was uniform and enhanced scanning showed a light strengthening of signal. A brain MRI of the man showed a well-defined mass measuring 4 cm in diameter invading the right ventricle. The mass showed low signal intensity on right T1-weighted images and high signal intensity on T2-weighted images. Histopathologically, the tumors were rich with significant and uniform proliferation of long spindle cells, which were arranged in fasciculate and knitted patterns with bipolar and spindled processes focally forming perivascular pseudorosettes. The cell nuclei showed minor pleomorphism. A few tumor cells centered on the vessels resulted in a nuclear-free zone, which was characteristic of ependymoma. Immunohistochemically, the tumor cells were positive for GFAP, EMA, and CD99, but negative for S-100. A review of the cases reported in the literature shows that tanycytic ependymoma occurs more often in the spinal cord. As it can resemble pilocytic astrocytoma and schwannoma, tanycytic ependymoma should be differentially diagnosed to exclude some of the benign spindle cell tumors of the central nervous system.     

Intravenous tufted angioma

A case of a rare vascular tumor, intravenous tufted angioma, is described. A 51-year-old Japanese man presented with a 12x8 mm solitary reddish nodule on the right foot, which had been found at birth. Histologically, the tumor was confined to a malformed vein and was characterized by nodular aggregates of plump cells. The aggregates showed a compact proliferation of round cells, including capillary-forming cells. Venous angiomatous areas were also observed. No multinucleated giant cells were seen. Immunohistochemically, the capillary-forming cells in the aggregates and the endothelial cells in the angiomatous areas were positive for endothelial markers (factor VIII-related antigen, CD31, CD34). Pericyte-like cells expressing alpha-smooth muscle actin and muscle actin, and macrophage-like cells, which stained for factor XIIIa, were intermingled in the cellular aggregates. Flow cytometric analysis showed diploidy. The tumor may be a hamartomatous lesion modified by secondary reactive changes, and it may represent a distinctive clinicopathological entity that is closely related histogenetically and perhaps pathologically to tufted angioma and the recently described "giant cell angioblastoma".     

Plexiform fibrohistiocytic tumor. Report of a case involving preoperative aspiration cytology and immunohistochemical and ultrastructural analysis of surgical specimens.

A typical case of plexiform fibrohistiocytic tumor (Enzinger and Zhang) occurring in the skin and subcutis of the abdominal wall in a 7-year-old girl is reported. Preoperative fine-needle aspiration cytology revealed a benign lesion with fibroblastic-histiocytic features which also contained bi- and multinucleated giant cells. The surgical specimen showed a tumor with multiple small nodules within fibrous septa; these nodules were composed of spindle cells and epithelioid cells and contained scattered multinucleated osteoclast-like cells. The tumor cells showed ultrastructural and immunohistochemical features of myofibroblasts and histiocyte-like cells. Thus, there was an abundance of lysosomes, prominent filopodia and bundles of thin cytofilaments along the cytoplasmic border, as well as immunoreactivity for alpha-smooth-muscle-specific actin, alpha-1-antitrypsin and alpha-1-antichymotrypsin. Ultrastructurally there were tumor cells exhibiting features of histiocytes which also contained bundles of actin of smooth muscle type. The presented case of plexiform fibrohistiocytic tumor appears to be composed of a rather peculiar cell form, somewhere between myofibroblasts and histiocytes.     

Metaplastic Breast Carcinoma Invading Chest Wall

Metaplastic breast carcinoma refers to a heterogeneous group of neoplasms in which the typical glandular growth pattern of the tumor undergoes metaplasia, either epithelial or stromal. A 59-year-old woman presented with a breast mass that recurred in 1 year and showed invasion of the chest wall. Histological sections of both the tumor and the recurrence showed a tumor composed predominantly of stromal spindle cells with neoplastic epithelial ducts. Squamous metaplasia was seen in some ducts. Immunohistochemical staining showed positive cytokeratin and epithelial membrane antigen staining of the epithelial cells. Smooth muscle actin, S100, and vimentin were diffusely positive in the stromal cells. Electron microscopy of the original lesion showed cells with squamous epithelial and smooth muscle characteristics, and other cells that formed lumens into which microvilli projected. Electron microscopy of the recurrent lesion showed primarily spindle-shaped cells with abundant tonofilaments in the perinuclear cytoplasm, desmosomes with associated tonofilaments, filaments with focal densities, often aligned parallel with the cell membranes, surface attachment plaques, and fragments of basement membrane. Pinocytotic vesicles were rare. These metaplastic cells are derived from myoepithelial cells which are multipotential and able to differentiate into epithelial or stromal cells.     

Cutaneous myofibroma

The clinical and pathologic features of an acquired skin tumor, which histologically is identical to infantile myofibromatosis, are presented. Thirty-four cases were studied, 26 of which were in patients over 14 yr of age. The median age was 36. None of the lesions was associated with recurrence after surgery, and metastases were not observed. Microscopically, the lesions were relatively well circumscribed and had a characteristic biphasic pattern which centrally showed features of vascular tumors, including hemangiopericytoma or glomus tumor. Ultrastructural examination identified cells with features of myofibroblasts, glomus cells, and pericytes. Immunoreactivity for muscle actin was strongly and diffusely positive in all tumors. The clinical significance of this entity lies primarily in its recognition as a distinctive benign neoplasm, most probably of vascular origin. The occurrence of this lesion is not limited to children; it may be an acquired lesion, and it may be found in the skin as well as soft tissue.     

Solitary fibrous tumor of the pancreas

A 67-year-old woman was found to have an incidental pancreatic mass on computed tomographic examination of her abdomen in the course of investigation of hematuria. The radiologic features were of a hypervascular mass in the uncinate process of the head of the pancreas, and a preoperative diagnosis of a neuroendocrine tumor was favored. A Whipple procedure was performed. The uncinate process contained a 2.6-cm well-circumscribed mass. Histologic evaluation showed a lesion composed of alternating hypercellular areas made up of spindle-shaped cells and hypocellular areas with hyalinized, keloidal-like fibrous tissue. Occasional dilated vascular channels and entrapped pancreatic tissue were present within the lesion. Immunohistochemistry showed the lesion to be CD34, CD99, and bcl-2 positive. No evidence of atypia was noted, and the overall impression was of a benign solitary fibrous tumor of the pancreas. This is an unusual primary spindle cell neoplasm of the pancreas and should be considered in the differential diagnosis of all spindle cell lesions that occur in the pancreas.     

Case of an inflammatory fibroid polyp of the cecum

An inflammatory fibroid polyp (IFP) is a rare benign lesion, originating in the submucosa of the gastrointestinal tract. It is histopathologically characterized by distinctively arranged fibrous connective tissue and blood vessels with inflammatory cell infiltration. It typically arises in the stomach and small intestine but also arises infrequently in the colon. This report describes a case of IFP of the cecum. A 63-year-old woman presented with persistent bloody stool for more than 1 month. Colonoscopy revealed a polypoid lesion, measuring 2.5 cm in diameter and 4 cm in length, with a thick pedicle in the cecum. Histopathological examination of the biopsy specimen showed hyperplastic changes of the mucosa. The lesion was diagnosed to be a submucosal tumor. We concluded that endoscopic mucosal resection would be difficult because the polyp showed signs of infiltration into the submucosa. Furthermore, the possibility of malignancy could not be ruled out. Laparoscopy-assisted ileocecal resection with lymphnode dissection was performed after the patient's informed consent was obtained. The lesion was finally diagnosed to be IFP on the basis of histopathological examination of the resected specimen. Immunohistochemical staining of the spindle-shaped cells, which were present around the small vessels in the stroma of the tumor, showed that the tissue expressed vimentin but not alpha-smooth muscle actin, desmin, S-100, c-kit or CD 34. IFP is difficult to diagnose without the recognition of its clinical and pathological characteristics. It is also important to determinate the depth of the lesion before selecting the therapeutic method.     

Solid pancreatic hamartoma

A case of solid pancreatic hamartoma in a 58-year-old Japanese woman is presented. She had no symptoms, and a pancreatic mass was incidentally found on screening ultrasonography 4 months before admission. The patient was not alcoholic and had no history of pancreatitis. Physical examination and laboratory data were unremarkable. Preoperative imaging demonstrated a nodule in the body of the pancreas, measuring 2.0 cm in maximum diameter, which showed marked delayed enhancement during dynamic CT. The patient underwent a distal pancreatectomy under the preoperative diagnosis of pancreatic endocrine tumor and had an uneventful postoperative course. A well-demarcated solid nodule, 1.9 cm in diameter, was evident in the body of the pancreas. Microscopically, the lesion was composed of non-neoplastic, disarranged acinar cells and ducts embedded in a sclerotic stroma with elongated spindle cells, lacking discrete islets. The stromal spindle cells were immunoreactive for CD34 and CD117. The histological diagnosis was solid hamartoma of the pancreas. There was no recurrence 5 months after surgery. Herein is reported a case of solid hamartoma of the pancreas and review of the literature. A search through the literature found only two cases of solid hamartoma of the pancreas, among the 14 cases previously reported as pancreatic hamartoma.     

Immunohistochemical studies of cell differentiation in a juxtaglomerular tumor

A renal tumor removed from a 29-year-old woman, who recently had had onset of primary hypertension, showed the histologic and ultrastructural features of a renin-secretory juxtaglomerular cell tumor. The expression of Factor VIII-related antigen (FVIII-RAG) was evaluated to identify endothelial differentiation of tumor cells in this lesion. Control tissue and vessels within the tumor showed normal expression of FVIII-RAG, but tumor cells as well as normal juxtaglomerular cells showed no localization of this antigen, which suggests the nonendothelial nature of these specialized cells.     

Endoscopic ultrasound guided fine-needle aspiration diagnosis of duodenal high grade neuroendocrine carcinoma underlying a villous adenoma: report of a case

Endoscopic ultrasound guided fine-needle aspiration biopsy is a reliable and accurate method for the diagnosis of submucosal lesions of the gastrointestinal tract. We report the cytopathologic findings of a case of duodenal high-grade neuroendocrine carcinoma in a 68-year-old woman who presented with melena and marked anemia, 45 years after kidney transplantation. Imaging studies performed in the work-up of melena showed a duodenal mass, which on endoscopy proved to be an exophytic, villous duodenal lesion, 3 cm from the ampulla. Forceps biopsy of the exophytic lesion showed a villous adenoma. Endoscopic ultrasound additionally revealed an underlying submucosal lesion and EUS-guided fine needle aspiration of this submucosal mass and of the enlarged mesenteric lymph nodes was diagnostic of a high-grade neuroendocrine carcinoma. The aspirates showed abundant cellularity with tumor cells arranged in sheets and occasional loose clusters. The neoplastic cells had a moderate amount of pale cytoplasm and large round to oval hyperchromatic nuclei with focally prominent nucleoli. Mitoses, apoptotic bodies and necrotic debris were also present. The tumor cells were strongly and diffusely positive for cytokeratin AE1/AE3, synaptophysin and chromogranin and showed a very high proliferative fraction on Ki67 staining, supporting the diagnosis of a high-grade neuroendocrine carcinoma. This is to our knowledge the first case of high-grade neuroendocrine carcinoma of the duodenum diagnosed by EUS-FNA. This case also emphasizes the diagnostic value of EUS-FNA sampling of the submucosal and intramural component of villous tumors of the gastrointestinal tract when mucosal forceps biopsies show only benign findings.     

An autopsy case of neoplastic angioendotheliosis mainly affecting the lung and presenting with atypical cells in peripheral blood]

Neoplastic angioendotheliosis (NAE) is a rare condition characterized by proliferation of atypical cells in small vessels of various organs. In the past, atypical cells seen in this condition were considered to originate from vascular endothelial cells. However, recent immunohistochemical studies have established that the tumor cells in this condition mainly originate from B-lymphocytes. NAE is likely to affect the central nervous system and skin. Cases of NAE with a primary lesion in the pulmonary vessels are rare. One such rare case of NAE was recently encountered by us. The patient was a 77-year-old woman who was admitted to our department because of fever and interstitial shadow in both lungs. After admission, fever did not subside, and the pulmonary shadow became more marked. Laboratory tests upon admission disclosed elevated LDH. Of isozymes, LDH2 and LDH3 were high. Anemia and thrombocytopenia gradually became aggravated, and atypical cells with large basophilic cell body and irregularly shaped nucleus subsequently appeared in peripheral blood. Thereafter, the patient's general condition worsened rapidly and she died about 5 months after admission. Post-mortem examination revealed pleomorphic atypical cells filling the lumen of small vessels in various regions of the body. This finding was particularly marked in pulmonary vessels. With various immunohistochemical stains, these cells were identified as cells of B-lymphocyte origin.     

Colonic carcinoid metastatic to the breast

Metastatic tumors of the breast are uncommon. Breast metastases from nonmammary malignant neoplasms are rare, accounting for approximately 2% of all breast tumors. We report the case of an ileal carcinoid tumor metastatic to the breast 10 years after the initial diagnosis. A 53-year-old woman presented to our clinic with a palpable breast lump. The mammogram was nonspecific. A lumpectomy was performed that, on frozen section, showed a neoplastic lesion. Permanent sections showed that the tumor was composed of sheets of small uniform cells divided into lobules by delicate vascular septa. Immunohistochemical analysis showed that the lesional cells were strongly positive to the neuroendocrine marker panel of antibodies: chromogranin A, neuron-specific enolase, synaptophysin, serotonin, and low-molecular-weight keratin. The lesional cells were negative to cytokeratins 7 and 20, estrogen and progesterone receptors, carcinoembryonic antigen, and c-Erb-B2 antibodies. The presence of pleomorphic neurosecretory-type granules within the cytoplasm of the tumor cells by ultrastructural analysis strongly suggested a metastatic lesion from a midgut carcinoid. A detailed review of the patient's medical records confirmed a right hemicolectomy for an ileal carcinoid with lymph node and omental metastases that had been performed elsewhere 10 years earlier. A detailed pathologic analysis of this lesion by light microscopy, along with histochemical, immunohistochemical, and ultrastructural analyses, aided in confirming the metastatic nature of the current breast lesion.