Pritilata Rout

Paraneoplastic multicentric reticulohistiocytosis: A clinicopathologic challenge

Multicentric Reticulohistiocytosis (MRH) is a rare, systemic non-Langerhans cell histiocytosis (non-LCH) with prominent joint and skin manifestations. It is mostly self limiting. However, 15-30% of the cases are associated with malignancy and carry a poor prognosis. We report the case of a 42-year-old man who presented with multiple reddish-brown papules that on biopsy showed aggregates of oncocytic histiocytes with several multinucleate giant cells. Immunostains were positive for CD 68, CD 45 and were negative for S-100, CD1a. An impression of multicentric reticulohistiocytosis (MRH) was made, with the recommendation to screen for malignancy. Electron microscopy of the skin lesions showed features consistent with non-Langerhans cell histiocytosis. The patient was later diagnosed with acute myeloid leukemia at a follow-up visit several months later. Thus, it appears prudent to screen and follow-up adults with MRH, to identify an underlying malignant condition.

Lymphocytic thrombophilic arteritis: An enigma

A 55-year-old woman presented with a 5-year history of livedo racemosa on her limbs. Histology showed vasculitis of medium-sized arteries with a circumferential, hyalinised, intraluminal fibrin ring. Her laboratory investigations did not indicate any underlying systemic disease. The findings were consistent with lymphocytic thrombophilic arteritis (LTA), alias macular arteritis, which is a recently described entity. The importance of LTA lies in the fact that it is a close clinical and microscopic mimic of polyarteritis nodosa (PAN). LTA is believed to be a distinct entity by some and as a form of PAN by others. We have discussed this case in our report.

Plasmacytoma of larynx--a case report.

Plasma cell myeloma, the most common plasma cell neoplasm, is characterized by the presence of multiple lesions in the bone marrow. A single isolated lesion may occur either in bone (solitary plasmacytoma of bone) or in soft tissue (extramedullary plasmacytoma). Most cases of extramedullary plasmacytoma occur in the head and neck region. The diagnosis is established by histopathology and immunohistochemistry. A detailed evaluation for lesions at other sites is recommended as extramedullary plasmacytoma treated by radiation therapy has better survival rates than plasma cell myeloma, which is treated by chemotherapy. A case of plasmacytoma of the larynx is presented highlighting clinical and histological features with a review of literature.

Comparison of bacillary index on slit skin smear with bacillary index of granuloma in leprosy and its relevance to present therapeutic regimens

Background: As the world moves toward elimination of leprosy, persistence of infective cases in endemic pockets remains a significant problem. The use of clinical criteria to decide the paucibacillary (PB) versus multibacillary (MB) regimens has greatly simplified therapy at the field setting. However, a small but significant risk of under-treatment of so-called “PB” cases which actually have significant bacillary load exists. This study was undertaken to assess this risk and compare two methods of assessment of bacillary load, namely bacillary index on slit skin smear (BIS) versus bacillary index of granuloma (BIG). Aims: To compare BIS with BIG on skin biopsy in consecutive untreated cases of leprosy. Materials and Methods: This prospective study was conducted over a period of 12 months, wherein new untreated patients with leprosy were consecutively recruited. After a thorough clinical examination, each patient underwent slit skin smear (SSS) where the BIS was calculated. The same patient also underwent a skin biopsy from a clinical lesion where, the BIG was calculated. SSS and skin biopsy for BIS and BIG respectively were repeated for all patients at the end of therapy for comparison. All patients received therapy according to World Health Organization-Multidrug Therapy Guidelines. Results: The BIG was positive in all cases where the BIS was positive. Significantly, BIG was positive in three cases of borderline tuberculoid leprosy with <5 lesions who received PB regimen, whereas the BIS was negative in all three cases. Conclusion: This study suggests that BIG may be a better indicator of the true bacillary load in leprosy as compared to BIS. Its role in management is significant, at least in tertiary care centers to prevent “under-treatment” of so called PB cases, which may actually warrant MB regimens.

Feasibility of Axillary Reverse Mapping and Clinicopathological Features Predicting ARM Node Metastasis in Breast Cancer—a Pilot Study

The axillary reverse mapping (ARM) technique has been described as an attempt to map and preserve the upper extremity lymphatic drainage during axillary lymph node dissection (ALND) and/or SLNB. This technique is based on the hypothesis that the lymphatic pathway from the upper extremity is not involved by metastasis from primary breast cancer. The ARM node/s however, has been found, in various studies, to be involved with metastatic foci in patients with extensive axillary lymph node metastases. Therefore, the oncological safety of this procedure has not yet been determined. In this pilot study, we assessed the ARM node intraoperatively for various parameters and compared it to final HPR, to try and determine the oncologic safety of preserving the ARM node. Seventy-two breast cancer patients were screened for this prospective pilot study which was planned to recruit 20 patients. The study was initiated on May 2014, 20 patients were recruited till July 2015. Eligibility criterion was as follows: patients requiring primary axillary lymph node dissection based on a clinically positive axilla. Forty-five patients were ineligible because they had either received neoadjuvant chemotherapy or underwent previous axillary surgery or axillary radiation (exclusion criteria). Seven patients refused to give consent. ARM node identification rate was 75%. The most common location of the ARM node was lateral to the latissimus dorsi pedicle (42.10%), none of them being malignant. None of the oval or firm nodes were malignant. Tumor deposits were identified in 13%. Fine-needle aspiration cytology (FNAC) had 100% specificity, 94.4% negative predictive value, 100% positive predictive value, and 50% sensitivity. ARM is feasible using blue dye alone, with an acceptable identification rate. Location, consistency, and intraoperative FNAC of the ARM node, put together, may be reliable parameters to predict involvement of the ARM node with metastasis.

Metaplastic carcinoma of breast and neurofibromatosis 1: A rare association

Neurofibromatosis 1 (NF1) patients are generally at higher risk of developing common malignant tumors such as brain and soft tissue tumors. These patients are 5-fold increased the risk of developing breast cancer by the age of 50 years after that the risk remains the same. The most common reported cancer is infiltrating duct carcinoma. We report a case of 61-year-old female with NF1 presented with pain and breast lump for the past 2 months. On mammography, a retroareolar solid cystic lesion measuring 32 mm × 30 mm × 30 mm was noted which was definitive for malignancy. Right-modified radical mastectomy with axillary lymph node dissection was done which revealed a neoplasm composed of glandular and squamous components. Immunohistochemically, these cells were triple negative for estrogen receptor, progesterone receptor, and human epidermal growth factor receptor 2-neu and expressed markers of basal cell differentiation. The final pathological staging was T2N0M0. This is the fourth case report in the English literature with such association. In this case report, we discussed the importance of breast cancer screening in such patients along with a review of the literature.

Clinicopathological study of recently added glioneuronal tumors

Background: Glioneuronal tumors are pathologically heterogeneous group of tumors containing both glial and neural components or glial tumors with neural differentiation. In the year 2007, three new entities have been added to the repertoire of glioneuronal tumors by the World Health Organization (WHO) which include papillary glioneuronal tumor (PGNT) (WHO Grade I), rosette-forming glioneuronal tumor of the fourth ventricle (RGNT) (WHO Grade I), and glioneuronal tumor with neuropil-like islands (GTNIs) (WHO grade II/III). The present study summarizes the clinical and neuropathological features of these three glioneuronal neoplasms. Materials and Methods: This study included seven cases of newer glioneuronal tumors (four cases of PGNT, two cases of RGNT, and one case of GTNI) which were reviewed. Results: The clinical presentations (patient characters), radiology, squash preparations, histology, and immunohistochemical findings of these cases are discussed including some of the morphological variations and follow-up. Conclusion: Glioneuronal tumors show considerable morphological and clinical diversity with some unique features. As these neoplasms are low grade and well manageable, the knowledge of their clinical presentation and histological diagnosis is essential for treatment. The present study is an attempt toward this.

An unusual penile spindle cell malignancy

Primary non-epithelial malignancies of the penis are rare. We report a patient with an unusual presentation of an unexpected spindle cell malignancy at this site. A 35- year-old man presented with a mass in the shaft of the penis with inguinal lymphadenopathy. A malignant spindle cell neoplasm was seen involving the corpora, consistent with leiomyosarcoma. Immunohistochemically, the tumour was positive for S100, HMB-45 and negative for SMA. A final diagnosis of amelanotic spindle cell melanoma was made. This case is being presented to reiterate the importance of including melanoma in the differential diagnosis of spindle cell sarcomas at uncommon sites.