F-18 Fluorodeoxyglucose Positron-Emission Tomography/Computed Tomography Image of Rare Case of Phaeohyphomycosis Causing Osteomyelitis of Scapula in a Postrenal Transplant Recipient.

Phaeohyphomycosis belongs to a heterogeneous group of fungal infections, originally described by Ajello et al. as mycoses, whose etiologic agents develop in host tissue as dark-walled, septate mycelial elements. Disseminated infections occur in immunocompromised patients, involving the paranasal sinuses, eyes, central nervous system, lymph nodes, and bone. We present here an interesting image of 18F fluorodeoxyglucose positron-emission tomography/computed tomography showing scapula osteomyelitis caused by phaeohyphomycosis.

Atypical MYC rearrangement pattern of 3' deletion and 5' amplification along with independent IGH rearrangement: A case study.

A spectrum of Cellular homolog of the v-myc oncogene (cMYC) alterations such as translocation, overexpression, mutation, and amplification plays an important role in lymphomagenesis, particularly in high-grade lymphomas, and are associated with prognostic significance. Accurate identification of cMYC gene alteration is important for diagnostic, prognostic, and therapeutic implications. With the application of different FISH (fluorescence in situ hybridization) probes that helped overcome the analytical diagnostic challenges as a result of variant patterns, we report rare, concomitant, and independent gene alterations in cMYC and Immunoglobulin heavy-chain gene (IGH) with detailed characterization of its variant rearrangement. Short-term follow-up post R-CHOP (rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone) therapy seemed to be favorable. Accumulation of many more literature studies on such cases with their therapeutic implications would lead to the categorization of these cases as a separate subclass in large B-cell lymphomas followed by molecular targeted therapy.

Primary Cutaneous Apocrine Carcinoma of the Scalp: A Rare Case Report.

Primary cutaneous apocrine carcinoma of the scalp is a rare adnexal sweat gland neoplasm. It is most commonly observed over eyelids and ear canals and is often confused with dermoid and epidermoid cysts, lipomas, cutaneous metastatic lesions, or basal cell carcinomas. We describe a 66-year-old male who presented with a midline scalp lesion. He was treated surgically with a wide local excision.

Diplopia as the presenting feature of acute lymphoblastic leukemia.

PURPOSE: To report a rare case of acute lymphoblastic leukemia presenting with diplopia to an ophthalmologist. OBSERVATIONS: A 29-year-old male patient presented to ophthalmology department with sudden onset of binocular diplopia in left gaze. Magnetic resonance imaging of brain and orbits revealed a thickened left medial rectus, with enhancement of right sixth nerve, bilateral third and fifth nerves. Bone marrow biopsy revealed acute lymphoblastic leukemia (ALL) with a Burkitt-type chromosomal translocation-t(8; 14) and the patient was started on chemotherapy. CONCLUSION AND IMPORTANCE: This was a case of incomitant esotropia worse with left gaze due to left medial rectus infiltration mimicking a left sixth cranial nerve paresis. Diplopia can be the only presenting symptom of ALL and it can involve either an extraocular muscle or a cranial nerve.

Impact of focality on prognostication of early and operable breast carcinomas of no special type.

BACKGROUND AND OBJECTIVES: Multifocal/multicentric (MF/MC) breast carcinomas are not uncommon and its prognostic significance debated. We attempted to analyze the association of focality and prognostic factors in operated pT1 and pT2 breast carcinomas of no special type (NST). MATERIALS AND METHODS: Retrospectively identified pathologically proven 124 unifocal (UF) and 49 MF/MC pT1 and pT2 breast carcinomas of NST over the past three years were compared in terms of clinical and pathological factors. RESULTS: The patients with MF/MC NST tumors were more likely to undergo radical surgery (P = 0.028). The tumors showed higher incidence of lymphovascular invasion (P = 0.024), perineural invasion (P = 0.046), ductal carcinoma in situ component (P < 0.001), higher number of positive axillary lymph nodes (P < 0.001), and higher anatomical staging (P = 0.048) when compared to the UF counterparts. Morphological intertumoral heterogeneity was noted in MF/MC tumors in 16 of 49 cases (32.65%). CONCLUSION: Most published studies on MF breast cancers have included all histological types and varying definitions. We included only pathologically defined stages and a single histological type to ensure "purity" of the groups. Higher anatomic staging and morphological interlesional heterogeneity suggest that early MF/MC tumors represent multiple primaries with a different biology. Careful consideration of features of each focus needs to be considered when deciding appropriate adjuvant therapy and for accurately prognosticating these patients. Immunohistochemical and morphological (grade) heterogeneity between the different foci may pose problems with "prognostic stage grouping" these tumors according to the American Joint Committee on Cancer staging system (8th edition).

Mucormycosis: an unusual masquerader of an endobronchial tumour.

Pulmonary mucormycosis is a life-threatening invasive fungal infection usually seen in the background of immunosuppression, haematological malignancies, or uncontrolled diabetes. Immunocompetent hosts can also be affected. Isolated endobronchial mucormycosis is rare with only a few cases reported in the literature. Here, we present a case of an endobronchial mass masquerading as a tumour that was later diagnosed as invasive mucormycosis by histopathological examination.

Generation and Partial Characterization of Rabbit Monoclonal Antibody to Pyroglutamate Amyloid-ß3-42 (pE3-Aß).

N-terminally truncated pyroglutamate amyloid-ß (Aß) peptide starting at position 3 represents a significant fraction of Aß peptides (pE3-Aß) in amyloid plaques of postmortem brains from patients with Alzheimer's disease (AD) and older persons with Down syndrome (DS). Studies in transgenic mouse models of AD also showed that pE3-Aß is a major component of plaques, and mouse monoclonal antibody to pE3-Aß appears to be a desirable therapeutic agent for AD. Since small peptides do not typically elicit a good immune response in mice, but do so favorably in rabbits, our aims were to generate and partially characterize a rabbit monoclonal antibody (RabmAb) to pE3-Aß. The generated RabmAb was found to be specific for pE3-Aß, since it showed no reactivity with Aß16, Aß40, Aß42, Aß3-11, and pE11-17 Aß peptides in an enzyme linked immunosorbent assay (ELISA). The isotype of the antibody was found to be IgG class. The antibody possesses high affinity to pE3-Aß with dissociation constant (KD) for the antibody of 1 nM. The epitope of the antibody lies within the sequence of pE3-FRHD. In dot blotting, the optimal detection of pE3-Aß was at an antibody concentration of 0.5 µg/ml. The threshold of pE3-Aß detection was 2 fmol. The antibody was sensitive enough to detect 10 pg/ml of pE3-Aß in sandwich ELISA. pE3-Aß was detected in AD and DS brain extracts in ELISA and immunoblotting. Immunohistological studies showed immunolabeling of plaques and blood vessels in brains from patients with AD, and DS showing AD pathology. Thus, the antibody can be widely applied in AD and DS research, and therapeutic applications.

Neuroectodermally Derived Neoplasm of Tonsil: Difficulty in Histomorphological Diagnosis.

Esthesioneuroblastomas are rare neuroectodermally derived neoplasms occurring in the upper nasal septum or cribriform plate. They have been rarely reported in other sites, when they are called ectopic esthesioneuroblastomas. Due to the scarcity of reports, publications and molecular/genetic studies of these tumours, their diagnosis is likely to be missed when they are ectopic. Similar to the case report in this journal by Zahedi et al., we had a case of neuroectodermally derived neoplasm of the tonsil, which was difficult to diagnose, due to the site of occurrence being unknown for these tumours.

'Ease of interpretation' of cytological smears stained with modified ultrafast papanicolaou stain: Interobserver agreement and reproducibility.

BACKGROUND: Since its inception in 1995, the Ultrafast Papanicoloau (UFPAP) cytological stain has undergone a number of modifications to suit the local availability of reagents and cost in different set ups. However, the reported results have been uniformly encouraging. We designed a study to investigate the inter-observer agreement in 'perceived ease of interpretation' of cytological smears stained with Modified Ultrafast Papanicoloau stain (MUFPAP). METHODS: After a small pilot study, we prospectively stained air-dried fine needle aspirate smears (FNACs) and Body Fluid smears with the standardized MUFPAP stain. The MUFPAP stained slides were evaluated in tandem with other routine cytological stains as well as independently by two pathologists. Two rater kappa was used to determine the agreement. RESULTS: The study included 93 fluids and 34 FNACs. A vast majority of the cases stained with MUFPAP were rated 'better' than the routine stains in terms of 'overall ease of interpretation' with considerable agreement. The agreement tended to be better for FNACs than fluid specimens. Cases with malignant pathology demonstrated a perfect agreement (kappa = 1) between the raters in terms of 'overall ease of interpretation' (91.7% cases were rated 'very good' by each pathologist) when compared to cases with benign pathology (kappa = 0.52). Nuclear characteristics were appreciated with a better agreement than other parameters. CONCLUSION: Modified UFPAP stain appears to be quick, reliable, cost-effective alternative in cytology, especially for detecting malignant cells in smears with low cellularity. Its specific advantage is robust nuclear staining against a clear background.

Occult invasion of sternothyroid muscle by differentiated thyroid cancer.

PURPOSE: To determine the incidence of occult invasion of sternothyroid by differentiated thyroid cancer (DTC) and identify clinico-pathological features associated with the same. METHODS: Retrospective study of a consecutive series of DTC patients undergoing surgery, with preoperative ultrasound showing no evidence of strap muscle invasion. All had en bloc excision of sternothyroid muscle along with thyroidectomy. Incidence of microscopic invasion of sternothyroid and clinicopathologic features associated with the same, were studied. RESULTS: A total of 76 patients with DTC (2010-2014) were identified, of whom 62 met the inclusion criteria and were included in this study. Of these, 22 (36%) had no extrathyroidal extension (ETE), 30 (48%) had minimal ETE without sternothyroid invasion and 10 (16%) had minimal ETE with microscopic sternothyroid invasion. The mean tumor sizes of the three sub-groups were 1.9, 3.1 and 4.9 cm, respectively, with a significant difference between no ETE and sternothyroid invaded sub-groups (p = 0.03). Out of the 40 cases with minimal ETE, 3 (7.5%) had positive tumor microscopic margin. Retaining sternothyroid in situ would have theoretically increased this proportion to 27.5%. Over a median follow-up of 52 months, 58 (94%) patients remained structurally disease free, with only 1 local recurrence. CONCLUSION: Occult invasion of sternothyroid muscle occurred in 16% of DTC in this series. Excision of the muscle en bloc with thyroidectomy, particularly in larger tumors, may confer benefit in accurately staging the disease, encompassing occult ETE and achieving clear microscopic margins.

Syringocystadenocarcinoma papilliferum with coexisting trichoblastoma: A case report with review of literature.

We present a unique case of syringocystadenoma with malignant transformation into syringocystadenocarcinoma papilliferum and coexisting trichoblastoma arising de novo in a scalp swelling of long duration in a 78-year-old woman. Syringocystadenocarcinoma papilliferum was described by the World Health Organization as an extremely rare malignant adnexal tumor of apocrine origin with varied histology with some cases reported in literature.

Synchronous Malignant Phyllodes Tumor with Skin Ulceration and Invasive Carcinoma as Collision Tumor.

Phyllodes tumor is a rare fibroepithelial biphasic tumor of the breast composed of hypercellular mesenchymal stroma and double-layered epithelial component, arranged in clefts with leaf-like projections. Phyllodes tumor with coincidental presence of invasive carcinoma or in situ ductal carcinoma in the same or distinct breast is a rare occurrence, documented by some reports. Invasive carcinoma can be seen within or outside the phyllodes tumor. Skin ulceration by malignant phyllodes tumor with coexisting invasive carcinoma as collision tumor is extremely rare. Here, we report an extremely rare presentation of malignant phyllodes tumor as a giant fungating mass with distinct invasive carcinoma in the same breast in a 51-year-old female.

Squamous Cell Carcinoma In situ of the Cervix with Superficial Intraepithelial Extension to the Endometrium of Lower Uterine Segment: A Rare Presentation.

Carcinoma of the cervix is the most common malignancy in women in India. Squamous cell carcinoma accounts for more than 70% of the cervical malignancies. Carcinoma of the cervix generally invades the uterine wall by direct extension with or without parametrial involvement, however, rarely can spread superficially to the inner surface of the uterus replacing the endometrium. It is an uncommon phenomenon for an in situ lesion of the cervix to have contiguous superficial spread to the endometrium. We report a case of cervical in situ squamous cell carcinoma in a 45-year-old female with contiguous extension to the endometrium of the lower uterine segment.

Crystal-Storing Histiocytosis: Report of a Rare Case Presenting With Pathological Fracture of Femur. Is There More to the Entity?

Crystal-storing histiocytosis (CSH) is a rare histiocytic lesion, most often described in association with lymphoid malignancies, especially plasma cell myeloma or lymphomas associated with monoclonal gammopathy. A few cases have also been described in patients without an underlying lymphoid/plasmacytic neoplasm. The histiocytes are characterized by intralysosomal accumulation of crystals composed of whole or part of the immunoglobulin molecule. The pathobiology is largely unclear. It is a rare phenomenon and the available literature is restricted to case reports and a few case series. We present a case of a 70-year-old gentleman who presented with pathological fracture of left neck of femur secondary to CSH, a presentation so far unreported in the literature. Because of associated clinical features, a plasma cell dyscrasia was suspected and the workup, including bone marrow biopsy, yielded a diagnosis of plasma cell myeloma. Histological examination of the excised femoral head showed near complete replacement of the marrow spaces with sheets of histocytes rich in intracytoplasmic crystals and only occasional plasma cells. The peculiar presentation with pathological fracture of femur in the index case and predominant tumefactive lesions in the cases in the literature might suggest a possible neoplastic origin of this lesion.

Generation and Partial Characterization of Rabbit Monoclonal Antibody to Amyloid-ß Peptide 1-37 (Aß37).

Secreted soluble amyloid-ß 1-37 (Aß37) peptide is one of the prominent Aß forms next to Aß40, and is found in cerebrospinal fluid (CSF) and blood. Recent studies have shown the importance of quantitation of CSF Aß37 levels in combination with Aß38, Aß40, and Aß42 to support the diagnosis of patients with probable Alzheimer's disease (AD), and the value of antibody to Aß37 to facilitate drug discovery studies. However, the availability of reliable and specific monoclonal antibody to Aß37 is very limited. Our aims were: 1) to generate and partially characterize rabbit monoclonal antibody (RabmAb) to Aß37, and 2) to determine whether the antibody detects changes in Aß37 levels produced by a γ-secretase modulator (GSM). Our generated RabmAb to Aß37 was found to be specific to Aß37, since it did not react with Aß36, Aß38, Aß39, Aß40, and Aß42 in an ELISA or immunoblotting. The epitope of the antibody was contained in the seven C-terminal residues of Aß37. The antibody was sensitive enough to measure CSF and plasma Aß37 levels in ELISA. Immunohistological studies showed the presence of Aß37-positive deposits in the brain of AD, and Down syndrome persons diagnosed with AD. Our studies also showed that the antibody detected Aß37 increases in CSF and brains of rodents following treatment with a GSM. Thus, our antibody can be widely applied to AD research, and in a panel based approach it may have potential to support the diagnosis of probable AD, and in testing the effect of GSMs to target AD.

Giant solitary ancient schwannoma of the pleura masquerading as bronchopneumonia.

We report a 53-year-old female who presented with complaints of sudden onset breathlessness, cough with expectoration for 6 months, and a right lung base lesion in the chest X-ray, which was diagnosed and treated as bronchopneumonia by the family physician. High-resolution computed tomography of the chest was done which revealed a large heterogeneously enhancing mass in the right thoracic cavity. She underwent thoracotomy and was found to have a giant encapsulated and well-circumscribed mass arising from the pleura. Excision biopsy of the mass revealed it to be an ancient schwannoma. Ancient schwannoma of the pleura is a very rare entity. Complete surgical resection is curative.

Generation of Rabbit Monoclonal Antibody to Amyloid-ß38 (Aß38): Increased Plasma Aß38 Levels in Down Syndrome.

Secreted soluble amyloid-ß (Aß)38 is the second most prominent Aß form next to Aß40, and is found in cerebrospinal fluid (CSF) and blood. Recent studies have shown the importance of quantitation of CSF Aß38 levels in combination with those of Aß40 and Aß42 to support the diagnosis of Alzheimer's disease (AD), and other neurodegenerative diseases, and to facilitate drug discovery studies. However, the availability of reliable and specific Aß38 monoclonal antibody is limited. Our first aim was to generate and partially characterize rabbit monoclonal antibody (RabmAb) to Aß38. The antibody was specific to Aß38, since it did not react with Aß37, Aß39, Aß40, or Aß42 in ELISA or immunoblotting. The antibody was sensitive enough to measure Aß38 levels in plasma. Our second aim was to quantitate Aß38 levels in plasma from older Down syndrome (DS) persons and age-matched controls. Persons with DS (35 years and older) have neuropathological changes characteristic of AD. Studies have shown that plasma Aß40 and Aß42 levels are higher in older persons with DS than in controls. However, none examined Aß38 levels in DS. Our quantitation data showed that, like Aß40 and Aß42 plasma levels, Aß38 plasma levels were higher in DS than in controls. Longitudinal studies will determine whether plasma Aß38 levels in combination with levels of Aß40 and Aß42 are useful to predict early signs of AD in DS.