Chicken pox outbreak in the Intensive Care Unit of a tertiary care hospital: Lessons learnt the hard way.

Varicella-zoster virus (VZV) causes 2 clinically and epidemiologically distinct forms of diseases. Chickenpox (varicella) is the disease that results from primary infection with the VZV. Herpes zoster (HZ) results from the reactivation of VZV latently infecting the dorsal root ganglia. We are reporting an outbreak of varicella infection among the health care workers (HCWs) in the Intensive Care Unit (ICU) of a tertiary care hospital. We found transmission of varicella among eight HCWs of pulmonary ICU. They had a history of contact with a patient having HZ infection. Investigation of the outbreak was conducted as per guidelines. Better dissemination of information on disease transmission, isolation of infected patients inside the hospital, and adequate protection (including vaccination) for susceptible employees are important to prevent such outbreaks.

Primary cutaneous large B cell lymphoma masquerading as lupus vulgaris.

Primary cutaneous large B cell lymphoma, leg type is a rare and aggressive variant of cutaneous B cell lymphoma. It predominantly affects elderly women, with the lower limb being the most common site of presentation. The overall prognosis is poor, compared to other cutaneous B cell lymphomas. A 47-year-old man presented with a progressively enlarging nodule over the medial aspect of the left foot since 2 months. Clinical examination revealed a nodular plaque-like lesion with central ulceration that measured 7 × 7 cm, firm in consistency, and with ill-defined margins. The initial clinical diagnosis was lupus vulgaris. An incision biopsy was done, which on histopathology and immunohistochemistry revealed a rare diagnosis of primary cutaneous B cell lymphoma, leg type. The patient was started on chemotherapy; however, he succumbed to his illness about 1 year after the initial presentation. It is a rare type of cutaneous lymphoma, which may masquerade infectious disorders such as lupus vulgaris. A detailed histopathological and immunohistochemical analysis is essential for its correct diagnosis and management. Only a handful of cases of this rare condition are reported to date. This case has been reported in view of its rarity and unusual clinical presentation.

Cranial Mycetoma: A Rare Case Report with Review of Literature.

Mycetoma is a chronic granulomatous infection caused by fungi or bacteria, known as eumycetoma and actinomycetoma, respectively. Mycetoma commonly affects young males belonging to low socioeconomic strata, usually barefooted agricultural workers. It mainly affects lower and upper limbs presenting as a painless swelling with discharging sinus. Rarely, is it encountered in the intracranial location. The diagnosis relies on the clinical presentation and identification of the etiological agents within the tissue, by histology and special stains. It is important to specify the fungal or bacterial etiology, because the treatment of each is completely different. The management of such infections is challenging and should involve early diagnosis, the use of antibacterials or antifungals, and surgical removal of the lesion. To the best of our knowledge, only seven cases of intracranial mycetoma have been reported. The present case highlights the rarity of this lesion, thereby contributing to the existing literature and presenting its diagnostic implications.

Mucinous adenocarcinoma of renal pelvis in a young male: a diagnostic challenge.

Primary mucinous adenocarcinoma of the renal pelvis is an extremely rare tumor with only a handful of cases reported to date. Clinical and radiological features are not specific, and hence, histopathological examination holds the key for definitive diagnosis. This tumor has mainly been described in the elderly population, with less than five cases reported in individuals aged <35 years. Here, we report a case of primary mucinous adenocarcinoma of the renal pelvis in a young male. A 31-year-old male presented with a history of right-sided flank pain for the past year. On examination, he had right-sided costovertebral tenderness. Computed tomography (CT) scan revealed the presence of a hyperdense mass lesion in the right renal pelvis with severe hydronephrosis and cortical thinning. Because of the non-functioning status, right nephrectomy was performed. To our surprise, histopathology showed the presence of mucinous adenocarcinoma of the renal pelvis with carcinoma in situ of the ureter. This case describes a rare presentation of primary mucinous adenocarcinoma of the renal pelvis, and highlights the importance of histopathological examination in reaching the correct diagnosis.

Clinicopathological Concordance in 2216 Cases of Skin Biopsy over One Year: An Indian Experience.

Introduction To determine the spectrum of diseases and the level of clinicopathological concordance in skin biopsies received over a period of one year. Methods A total of 2216 skin biopsy cases received over a period of one year at a tertiary care center were retrospectively analyzed. The cases were further divided into further categories in levels of concordance based on the agreement between the clinical and histopathological diagnosis rendered. Results Of the cases, 61.01% showed clinicopathological concordance. Cases with a descriptive pathological diagnosis, not matching the clinical diagnosis, constituted 31.54%, whereas 4.02% of cases had a definitive pathological diagnosis, which was discordant with the clinical differentials; 3.29% biopsies were inadequate. Conclusion This study highlights the clinicopathological concordance in all the biopsies received from dermatology. It emphasizes the importance of skin biopsies in arriving at the diagnosis. However, it is a tool that must be used judiciously. Skin biopsies are also pivotal in flagging malignancies that may mimic benign lesions.

Isolated collagenoma on the face: a rare occurrence.

Collagenomas are connective tissue nevi with hamartomatous proliferations of dominant dermal collagen. They can present as solitary or multiple inherited or acquired lesions over various body sites. The face is a rare site of collagenomas and, of the few cases reported in the literature, they have been seen more often on the scalp or on the plantar area. An extensive literature search did not reveal any cases of isolated collagenoma on the face. Herein we present the case of 22-year-old female with isolated collagenoma on the face. This case is being reported because of its unique location and rarity.

Juvenile Dermatomyositis: A Case Report and Review of Literature.

Juvenile dermatomyositis (JDM) is a systemic inflammatory disease involving children, which primarily affects the skin and the musculoskeletal system. The characteristic findings include Gottron papules, heliotrope rash, calcinosis cutis, and symmetric proximal muscle weakness. Histologically, it is characterized by the presence of lymphocytic vascular inflammation and endothelial swelling. Herein, we report a case of a 10-year-old girl of Indian origin, who presented to us with classical clinical and histological features of JDM.

Cluster of Differentiation 10 Positive Stromal Sarcoma of Breast: A Diagnostic Challenge.

Stromal sarcomas of the breast are a group of rare and heterogeneous tumors which mimic malignant phylloides tumor and metaplastic carcinoma histologically. These tumors have been reported in the literature mostly in form of small retrospective case series and case reports, making it difficult to characterize their exact histopathological spectrum and management strategy. Our patient was a 65-year-old female who presented with a 3-month history of a lump in the left breast. Examination revealed an immobile mass in upper and outer quadrant of left breast, 5 x 4 cm in diameter. Trucut biopsy revealed sheets of atypical spindle cells. She underwent a modified radical mastectomy. On histopathology, we found malignant mesenchymal tumor positive for vimentin and cluster of differentiation 10 (CD10). Thus, a rare diagnosis of CD10 positive mammary stromal sarcoma was established. The case has been reported for its rarity and to highlight the importance of a meticulous histopathological examination for excluding close differentials.

Collision of Malignant Melanoma and Squamous Cell Carcinoma in Rectum: First Report of a Rare Tumor.

The term collision tumor is used to describe two neoplasms occurring in the same anatomic location with juxtaposition of different tumor elements. Such a coexistence of tumors anywhere in the body is relatively rare. We report a case of 32-year-old female with collision tumor of rectum. The tumor showed two distinct histological patterns with predominant component consisting of malignant melanoma and a minor component of squamous cell carcinoma. The morphological picture of collision was further confirmed by specific immunohistochemical profile of the two tumors. Collision tumors of rectum are uncommon with most of the reported cases comprising adenocarcinoma and neuroendocrine tumors. To the best of our knowledge this is the first case of collision tumor of malignant melanoma and squamous cell carcinoma in the rectum.

Parathyroid carcinoma: an unusual presentation of a rare neoplasm.

Parathyroid carcinoma is an extremely rare malignant endocrine neoplasm that is very challenging in its diagnosis as well as its treatment. Clinically the disease is detected earlier in patients who present with hyperparathyroidism with signs of profound hypercalcemia. Differentiation between benign and malignant disease of the parathyroid is challenging both for the clinician and for the pathologist. Complete surgical resection at the time of first operation offers the best chance of cure. Even after radical excision which is the standard management, local recurrence and metastases are frequent. The disease usually has a slow indolent course and most patients suffer from complications of hypercalcemia rather than tumor invasion or metastasis. We report a case of a 31-year-old woman who presented with renal colic. Various hematological, biochemical and radiological investigations were performed and a slightly enlarged right parathyroid was found. A clinical diagnosis of parathyroid adenoma was made and a right parathyroidectomy was done. Intraoperatively the surgeon had no suspicion of malignancy but microscopically the lesion was malignant and a final diagnosis of parathyroid carcinoma was rendered based on the criteria of invasion. Since there is no gold standard, a multidisciplinary approach, including the entire clinical, biochemical, radiological and pathological profile of the disease aids in an accurate diagnosis. Here we are reporting a case of a functional parathyroid carcinoma presenting in a relatively young patient with all the biochemical and radiological investigations and intraoperative findings pointing towards a benign parathyroid disease.

Matrix-producing metaplastic breast carcinoma - a rare tumor with heterologous elements.

Metaplastic breast carcinomas are ductal carcinomas that undergo metaplasia into non-glandular growth patterns. They are very rare and account for less than 1% of all invasive breast carcinomas. Matrix-producing carcinoma is an extremely rare and aggressive subtype of metaplastic breast carcinoma that is characterized by a ductal carcinomatous component with direct transition to areas with cartilaginous/osseous differentiation without an intervening spindle cell element. It has a better prognosis than metaplastic carcinoma. Even though these tumors are composed of a mixture of infiltrating ductal carcinomas and areas of heterologous stroma, each of which behaves aggressively individually, these composite tumors have a better 5-year survival rate with rare nodal metastasis. Immunohistochemically, they are positive for keratin, epithelial membrane antigen and S100. The tumor, which is matrix-producing, is S100-reactive and nonreactive for cytokeratin. They are usually hormone receptor-negative. The average age of these patients is approximately 58 years. Since these tumors are usually triple-negative, chemotherapy after surgery is the mainstay of therapy, using either mastectomy or local excision. Our report highlights this rare entity in a 55-year-old female patient with matrix-producing metaplastic breast carcinoma. Its distinctive histological features and peculiar clinical behavior warrants clear knowledge about this unique entity.

Epidermodysplasia verruciformis: three case reports and a brief review.

Epidermodyplasia verruciformis (EV) is a rare heritable disease that is characterized by an increased susceptibility to infection with specific human papillomavirus (HPV) types due to a defect in cell-mediated immune response to HPV infection. Widespread infection with HPV is responsible for the development of wart-like lesions and pityriasis versicolor-like spots. These individuals have a lifelong risk of developing cutaneous malignancies, especially Bowen's disease and squamous cell carcinoma, mainly in sun-exposed parts. Being the first disease to correlate cancer and viral infection, EV serves as the cornerstone of the understanding of viral oncogenesis. We report three cases of EV, of which one patient subsequently developed Bowen's disease. The patients had multiple hypopigmented papules and plaques of varying sizes that started erupting in childhood and were mainly distributed over sun-exposed parts of the body. Histopathology of the skin biopsies was consistent with EV. One of these patients had started developing an ulcerated plaque over the left clavicle 2 years earlier. A biopsy from the clavicular region also showed histopathological features of Bowen's disease arising in EV. Hence, this clinical review discusses three cases of EV presenting in different age groups with detailed histopathological findings typical for EV.

Phaeohyphomycosis of the Face Masquerading as Basal Cell Carcinoma in an Immunocompetent Patient.

Pheohyphomycosis is a rare heterogeneous group of mycotic infections caused by dermatiaceous (phaeoid) fungi affecting the skin, subcutaneous tissue, and the central nervous system.Involvement of the face is extremely rare, and very few cases have been reported in India so far. We reporta case of phaeohyphomycosisin a 45-year-old female with 1-year history of a well- defined hypertrophic plaque over the right cheek advancing towards the forehead. The lesion was ulcerated with rolled-up margins; a provisional clinical diagnosis of basal cell carcinoma was given. Histopathology of the skin biopsy revealed numerous multinucleated giant cells and fungal hyphae with in and in between the giant cells. Various histochemical stains were used toconfirm the presence of fungal hyphae. Melanin pigment in the fungus was demonstrated with Masson's Fontana stain. Based on the histopathological and histochemical findings, a diagnosis of phaeohyphomycosis was given and it was concluded that the disease was more of a histopathological than clinical diagnosis. This case is being reported due to its unusual presentation and it also highlights the importance of histopathology in the diagnosis of this rare disease.

Choroid plexus carcinoma: report of two cases.

Choroid plexus carcinomas (CPCs) are rare malignant counterparts of choroid plexus papilloma which occur in infants and children with a predilection for the posterior fossa and have a poor prognosis. We report two cases of CPC diagnosed in a 5-year-old boy and a 12-year-old boy and discuss the clinicopathologic features.

Papillary meningioma: a rare but distinct variant of malignant meningioma.

BACKGROUND: Papillary meningiomas are rare meningeal tumors and are associated with aggressive clinical behavior as compared with other meningiomas. Because of their rare occurrence, they may pose a diagnostic dilemma to the unwary pathologist. We report a case of papillary meningioma in a 16-year-old boy. CASE PRESENTATION: A 16-year-old boy presented with complaints of headache, progressively diminishing vision and more recently generalized seizures. MRI revealed a large bifrontal meningioma which showed presence of a predominantly papillary pattern with areas of focal necrosis, frequent mitoses and bone invasion. He underwent radical excision of the tumor and is free from recurrence or metastasis at 15 months follow-up. CONCLUSION: Papillary meningiomas are rare but well recognized variants of meningioma. They need to be differentiated from other intracranial tumors with a papillary pattern. They are malignant, frequently show bone and parenchymatous invasion and have the potential for extracranial metastasis. Their timely recognition could prevent local and distant metastasis and the mortality or morbidity associated with it.