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Monotypic angiomyolipoma is usually found in the kidneys and is composed predominantly of epithelioid cells which show positivity for melanocyte and smooth muscle markers. It can pose a diagnostic challenge due to a range of differential diagnosis. We report the second case of monotypic angiomyolipoma of nasal cavity and first from India in a 54-year-old male who presented with a nasal polyp. Grossly the tumor was well circumscribed and un-encapsulated. Microscopy showed a large number of epithelioid cells mixed with a few spindle cells, varying sized blood vessels, and focal areas of adipose tissue. Immunohistochemistry was positive for smooth muscle actin (SMA) and human melanoma black (HMB-45) stains. It is important to identify this tumor as it can sometimes be mistaken for malignancy and only needs endoscopic resection.

A breast mass in a postmenopausal age woman is treated with a high index of clinical suspicion for malignancy. Myofibroblastoma (MFB) of the breast is a very rare benign stromal tumor, predominantly occurring in menopausal women and older men. Owing to its rarity, nonspecific radiology, cytomorphology, and many variants, it can be confused with other malignant and benign breast lesions and hence can be a source of diagnostic pitfall. We present a case of an MFB of the breast in a 55-year-old female, which was detected on a routine screening mammography. Fine-needle aspiration cytology was inconclusive. Final diagnosis was made by histopathology and immunohistochemistry examination. We report this case as the likelihood of encountering MFB has increased in recent years due to routine mammographic screening, and this lesion should be kept in the differential diagnosis of spindle-cell lesions of the breast.

Primary cutaneous large B-cell lymphoma (Bcl) is defined as a lymphoma composed of large cells constituting more than 80% of the infiltrate and absence of extracutaneous involvement after staging investigations. In the new World Health Organization/European Organization for Research and Treatment of Cancer classification, cutaneous Bcls with large cells are of three types - primary cutaneous large Bcl leg type (PCLBCLLT), primary cutaneous follicle center lymphoma diffuse type (PCFCLDT), and primary cutaneous large Bcls other (PCLBCLO). These three different types are distinct in terms of their clinicopathological features and survival. The PCLBCLO has intermediate features between those of PCLBCLLT and PCFCLDT. We present a case of PCLBCLO in a 57-year-old male who presented with a scalp swelling. Ultrasonography examination was suggestive of a sebaceous cyst. Computed tomography scan revealed the presence of an ill-defined hyperdense region in the soft tissue of the scalp region extending into the deeper layers of the scalp. Fine-needle aspiration cytology (FNAC) revealed the presence of atypical lymphoid cells. Diagnosis was confirmed by biopsy and immunohistochemistry. Patient received rituximab combined with doxorubicin, vincristine, cyclophosphamide, and prednisolone regimen with complete resolution of the lesion. We present this case for its rarity, the utility of FNAC in early diagnosis, and to discuss the differential diagnosis.

Vascular malformations (VMs) are structural malformations of vascular development causing soft tissue abnormality with functional and esthetic impairment. They are named by their predominant vessel type as arterial, venous, lymphatic or mixed types. VMs of the parotid gland are extremely rare and constitute a distinct entity of parotid pathology that requires specific diagnostic tools and management. Till 2013, only fifty cases of VMs of the parotid have been described in the literature. We present a case of a venolymphatic malformation of the parotid gland extending into the parapharyngeal space in a 21-year-old male who presented with a swelling on the left side of the face extending into the neck. Diagnosis was suggested by ultrasonography and computed tomography scan and was confirmed by magnetic resonance imaging examination. Complete surgical excision of the lesion was done with a favorable outcome. Diagnosis was confirmed based on histopathology and immunohistochemical studies.

Papillary cystadenocarcinoma (PCAC) is a rare salivary gland tumor characterized by a predominantly cystic growth that often exhibits intraluminal papillary growth without specific histologic features of other cystic salivary gland tumors. The preoperative cytological diagnosis can pose a diagnostic challenge as it has to be differentiated from other cystic papillary tumors such as mucoepidermoid carcinoma, papillary cystic variant of acinic cell carcinoma, and low-grade cribriform CAC. It is considered to be a low-grade malignant salivary gland tumor with an indolent biological behavior. We report a case of PCAC of the parotid in a 55-year-old male diagnosed on fine needle aspiration cytology. Although it showed mild atypia cytologically, on excision tumor showed vascular and perineural invasion with regional node metastasis indicating a wider morphologic spectrum than what is described. This prompted us to write a case report describing the cytological and histological features of this rare tumor and also discuss the diagnostic challenges.

Pemphigoid vegetans is a very rare type of bullous pemphigoid which usually affects the elderly and has not been reported in children. It shows a clinical resemblance to pemphigus vegetans but has distinct histological and immunopathological features of bullous pemphigoid. A 9-year-old girl presented with recurrent purulent and verrucous vegetating lesions on her forehead, groin and vulva along with scaling, crusted, bullous and purulent lesions on the eyelids, periorbital, periauricular, perioral region and lips. She had oral lesions and a cerebriform tongue. Though she showed clinical features of pemphigus vegetans, histology revealed a subepidermal blister with the absence of acantholysis. Direct immunofluorescence studies were suggestive of bullous pemphigoid. On clinicopathological correlation, a diagnosis of pemphigoid vegetans was made. She responded well to oral corticosteroids and dapsone therapy with complete resolution of the lesions.