Case 231: Retroperitoneal Adrenal Teratoma Presenting as Trichoptysis.

History A 24-year-old woman from a rural village presented with vague left hypochondrium pain and a cough for the past 2 years. She had a history of occasionally expectorating hairlike strands with her cough. Because the patient was from a rural area, she first consulted with the village priest, as she presumed her illness to be some supernatural phenomenon. The priest advised her to collect the strands for religious rituals ( Fig 1 ). She collected these strands for some time; however, because her cough worsened, she visited the hospital. General physical examination findings were within normal limits. On palpation, there was evidence of a vague lump in the left hypochondrial region. At ultrasonography (US) (images not shown), a large mass with heterogeneous echotexture was seen in the left suprarenal region; cystic areas and calcification were present. Chest radiography (images not shown) revealed bronchiectatic changes, with consolidation in the left lower zone. Results of a blood examination, including assessment of renal function, liver function, and complete blood count, were within normal limits. Unenhanced and contrast material-enhanced computed tomography (CT) images of the chest and abdomen were obtained.

A Novel cause of Massive Hepatosplenomegaly with Fibrosis in two children: Transient Infantile Hypertriglyceridemia.

Background: Transient infantile hypertriglyceridemia (TIH) is a syndrome of hypertriglyceridemia, fatty liver, and deranged liver functions with progression to fibrosis and cirrhosis. It is an autosomal recessive disorder caused by mutations in Glycerol-3-phosphate dehydrogenase 1 gene present on Chromosome 12q12-q13, and has been reported in Israeli Arab families with high consanguinity. TIH is suspected by high serum triglyceride levels and steatosis on liver biopsy; however, diagnosis is confirmed on clinical exome sequencing. Clinical description: We present two cases of TIH belonging to the indigenous Hindu, hilly population of Himachal Pradesh in North India with no history of either consanguinity or family history. Management and outcome: The parents of both the cases were counselled regarding the disease and importance of growth and lipid level monitoring. Conclusion: Though TIH is an extremely rare entity, awareness about it is required as it is a contributor to non-alcoholic fatty liver disease (NAFLD) in children. Any child presenting with hepatomegaly and elevated fasting triglyceride levels should be further investigated for TIH.

Matrix-producing mammary carcinoma: a rare breast tumor.

Matrix-producing carcinoma of the breast is a unique subclass of metaplastic carcinoma which is characterized by the existence of a ductal carcinomatous component with direct transition to areas showing cartilagenous or osseous differentiation, lacking an interspersed spindle cell component. This article reports one such rare case in a 50-year-old woman who had a right breast mass. f0 ine needle aspiration (FNA) smears showed abundant chondromyxoid extracellular matrix to which were variably admixed carcinomatous cells. Histological examination revealed a neoplasm composed of invave ductal carcinoma with a direct transition to chondrosarcomatous areas. The case is reported not only for its peculiar microscopic characteristics but also to highlight its better prognostic features and hence, the need for its recognition.

An Enigmatic Clinical Presentation of Plasma Cell Granuloma of the Oral Cavity.

Plasma cell granuloma is a rare benign lesion characterized by the infiltration of plasma cells; primarily occurring in the lungs. It is also seen to occur in the brain, kidney stomach, heart, and so on but its intraoral occurrence is a rarity. This case report represents one of the uncommon locations in the oral cavity affected by plasma cell granuloma, its clinical and histological features, and establishes the differential diagnosis with other malignant or benign disease entities and planning the treatment accordingly. This report discusses the diagnostic enigma and the associated terminology of plasma cell granulomas and reinforces the need for performing biopsy and a histopathological or immune histochemical study, irrespective of the clinical features and clinical diagnosis of the lesion. In this case a 52-year-old female, presented with gingival enlargement in the mandibular anterior region, treated by excisional biopsy. Histological evaluation revealed plasma cell infiltrates in the connective tissue. The immune-histochemistry revealed kappa and lambda light chains with a polyclonal staining pattern, which confirmed the diagnosis of plasma cell granuloma.

Fine needle aspiration cytology of breast carcinoma with neuroendocrine features--a case report with histopathological and immunohistochemical correlation.

Cytohistological and immunohistochemical features of a rare case of breast carcinoma with diffuse neuroendocrine features occurring in an elderly female patient is reported. Cytology demonstrated predominantly single and loose clusters of monomorphic plasmacytoid tumor cells that possessed moderate amounts of basophilic cytoplasm with eccentrically placed nuclei. These cells were also showing conspicuos rosette like formations. Histological examination showed a typical endocrine pattern with organoid nests and ribbons of well differentiated monomorphic cells with frequent pseudorosette formation resembling carcinoid tumor of gastrointestinal tract. Neuroendocrine differentiation was confirmed by immunohistochemical positivity for neuron specific enolase, synaptophysin and chromagranin.

Mucoepidermoid carcinoma arising in Warthin's tumor: a case report.

Malignant transformation of the epithelial component of Warthin's tumor is extremely rare. We describe our experience of mucoepidermoid carcinoma arising in Warthin's tumor of the parotid gland in a 35 year old female. The tumor removed from the parotid region was well encapsulated and histologically comprised ofmucoepidermoid carcinoma along with areas of Warthin's tumor. The pathogenesis and differential diagnosis of this rare occurrence have been discussed.

A rare case of secretory breast carcinoma in an elderly woman: correlation of aspiration cytology and histology.

Secretory carcinoma (SC) of the breast is an extremely rare neoplasm, often associated with a very good prognosis. It is still rarer in adults, where it is potentially more aggressive than in childhood. The present case refers to a 52 year old female with a progressively growing lump in the right breast. The cytological and histopathological findings were characteristic of secretory carcinoma. The differential diagnoses are discussed and the pertinent literature is reviewed.

Papillary thyroid carcinoma with nodular fasciitis-like stroma: A rare variant with distinctive morphology.

Papillary thyroid carcinoma (PTC) is a common malignancy with multiple variants, some of which are rarely encountered in routine surgical pathology practice. PTC with exuberant nodular fasciitis-like stroma or PTC with fibromatosis-like stroma is one such variant. This tumor is characterized by an abundant stromal component with an intervening epithelial component with the typical morphologic features of PTC. We describe gross and histopathological features of this rare variant of papillary carcinoma in a 38-year-old female and review the literature. We also discuss the importance of a thorough search for epithelial components within any fibroproliferative lesion of the thyroid and address the diagnostic difficulties created by the tumor's extensive stromal component. Given the rarity of this condition, the experience gained from the present case is a useful addition to the current knowledge on disease prognostication and management.

Well differentiated neuroendocrine tumor of the kidney: Report of a rare case with review of literature.

Neuroendocrine tumors (NETs) are uncommon tumors that exhibit a wide range of neuroendocrine differentiation and biological behavior. Primary NETs of the kidney, including carcinoid tumor, small cell carcinoma (SCC), and large cell neuroendocrine carcinoma (LCNEC) are exceedingly rare. Renal carcinoids are typically slow-growing tumors and pursue a variable clinical course. In contrast, SCC and LCNEC often present with locally advanced or metastatic disease and carry a poor prognosis. We herein report a rare cases of well-differentiated NET (carcinoid) in a 39-year-old male along with the immunohistochemical features. The rarity of these tumors poses a diagnostic and therapeutic challenge.

Intradural Eosinophilic Granuloma Invading Skull: Case Report and Review of the Literature.

Eosinophilic granuloma is a localized form of Langerhans cell histiocytosis, most commonly involving the skeletal system. Their origin from the dura is rare with only a handful of cases on record. We present one such rare case of an eosinophilic granuloma originating from the dura mater with secondary osseous invasion in an 11-year-old female child who presented with a swelling in the right parietal region. Magnetic resonance imaging demonstrated an enhancing mass with a wide dural attachment with a lytic lesion in the overlying skull. Right parietal extended craniotomy was done with the excision of mass from the dura. Histopathological features of mass were characteristic of eosinophilic granuloma which was confirmed by positive immunohistochemical staining for CD1a.

Primary apocrine carcinoma of an unusual site.

Primary apocrine carcinoma is a rare malignancy most commonly occurring in apocrine dense areas like axilla. There are only about 200 cases reported to date. We report a case of primary apocrine carcinoma present at an unusual site, that is, the arm. A wide local excision of the mass was done and was diagnosed as apocrine carcinoma on histopathological examination and was confirmed by immunohistochemistry. Wide local excision is the treatment required.

Invasive lobular carcinoma of male breast--a case report.

A 56 year old male presented with painless subareolar lump in the left breast was subsequently diagnosed as invasive lobular carcinoma. This case is reported in view of its rarity.

Focal eosinophilic myositis--a rare cause of pseudotumor.

Idiopathic focal eosinophilic myositis is a rare benign pseudotumor of skeletal muscle of unknown cause, characterized by focal eosinophilic infiltration of striated muscle. It can mimic a soft tissue sarcoma clinically and on gross examination of the specimen. Microscopic examination establishes the diagnosis. It is important to recognize this pseudosarcomatous lesion so as to spare the patient from anxiety.

Calcifying fibrous pseudotumor of the soft palate--a case report.

Calcifying fibrous pseudotumor has recently been described in the soft tissues. It is a rare benign lesion characterized by the presence of abundant hyalinized collagen with psammomatous or dystrophic calcification, and lymphoplasmacytic infiltrate. We report one such rare case in an 18 year old female with a nodular mass in the soft palate treated by complete resection. The mass had all the pathologic features of a calcifying fibrous pseudo-tumor.

Mucinous cystadenoma of the renal pelvis with malignant transformation: a case report.

A mucinous cystadenoma of the renal pelvis with malignant transformation is an extremely rare entity. We discuss one such rare occurrence in a 62 year old female who developed mucinous nephrosis due to marked retention of mucin produced by the tumor.

Rhabdoid meningioma lacking malignant features: Report of a rare case with review of literature.

We reported a case of meningioma with rhabdoid morphology but lacking histological features of malignancy in arising from the spinal cord in a 28-year-old male. The tumor showed light microscopic, immunohistochemical evidence of meningothelial differentiation together with diffuse areas exhibiting rhabdoid morphology. The rhabdoid areas were characterized by cells with large cytoplasmic eosinophilic inclusions and eccentric nuclei. Unlike most cases reported in the literature, this case lacked significant mitotic activity and other atypical features. The diagnostic and prognostic significance of this tumor entity is discussed along with a review of the literature.

Ganglioneuroma arising from the L5 nerve root: A rare case report.

Ganglioneuroma is a rare, benign, slow-growing, well-differentiated tumor consisting ganglion cells and Schwann cells. Ganglioneuromas originate from neural crest cells and can affect any part of the sympathetic tissue from the skull base to the pelvis. However, ganglioneuroma occurring in the nerve root is extremely rare. We describe a 44-year-old man with ganglioneuroma involving the right fifth lumbar nerve root.

Histopathological evaluation of placentas in IUGR pregnancies.

Gross and microscopic examination was conducted on one hundred placentas. These included twenty five normal controls and seventy five from intrauterine growth retardation (IUGR) pregnancies. Weight of the placentas from IUGR pregnancies were less than those of normal placentas. The incidence of infarction, intervillous fibrin deposition were much higher in IUGR placentas on gross examination. Highly significant increase in the incidence of infarction, intervillous fibrin deposition, stromal fibrosis and syncytial knotting were found in IUGR placentas compared to full term normal placentas on microscopic examination. The incidence of basement membrane thickening and cytotrophoblastic hyperplasis were also higher in IUGR placentas. All the major histologic findings pointed towards reduced blood flow to the placentas resulting in the restriction of blood flow to fetus. The information obtained from their examination can be a useful adjunct in planning and management of future pregnancies.