Pseudoangiomatous stromal hyperplasia: A clinical perspective and a brief review.

ABSTRACT: Pseudoangiomatous stromal hyperplasia (PASH) is a benign entity of the breast and typically found incidentally or as a palpable mass. It usually affects women in the reproductive age group. In men, it is associated with gynecomastia. While the exact etiology is unknown, PASH is considered hormonally responsive. The mammography and ultrasonography findings are nonspecific. Grossly, PASH is a well-circumscribed, firm, rubbery mass with solid, homogenous, gray-white cut surface. On histologic examination, it is characterized by the presence of open slit-like spaces in dense collagenous stroma. The spindle cells express progesterone receptors and are positive for vimentin, actin, and CD34. PASH lesions should be surgically excised if increasing in size or associated with symptoms, also in cases of suspicious imaging findings or other lesions are synchronously diagnosed that require excision. In other small incidentally detected asymptomatic cases observation with clinicoradiological follow-up can be considered. It is associated with a good prognosis with no increased risk to develop breast cancer. Hence, the operating surgeon and pathologist should be aware of this not so uncommon entity so that patients are not subjected to unnecessary excision and subsequent physical and mental morbidity associated with it. In this article, we present a case report and also provide a brief review of the sparse literature on PASH, discussing clinical presentation, etiology and pathogenesis, radiopathological features, differential diagnosis, and the implications for further patient management and prognosis.

Interobserver variation is a significant limitation in the diagnosis of Burkitt lymphoma.

CONTEXT: The pathology of classic Burkitt lymphoma (BL) remains a challenge despite being a well-defined entity, in view of the significant overlap with atypical BL and B-cell lymphoma intermediate between DLBL (diffuse large B cell lymphoma) and BL. They are difficult to be segregated in resource-limited setups which lack molecular testing facilities. This is further affected by interobserver variability and experience of the reporting pathologist. AIMS: The aim of our study was to quantitate variability among a group of pathologists with an interest in lymphomas (albeit with variable levels of experience) and quantitate the benefit of joint discussions as a tool to increase accuracy and reduce interobserver variability of pathologists, in the diagnosis of BL in a resource-limited setup. MATERIALS AND METHODS: A set of 25 non-Hodgkin lymphoma cases in which a diagnosis of BL was entertained were circulated to 14 participating pathologist within the Mumbai lymphoma study group. A proforma recorded the morphologic and immunohistochemical features perceived during the initial independent diagnosis followed by a consensus meeting for discussion on morphology and additional information pertinent to the case. STATISTICAL ANALYSIS AND RESULTS: The concordance was poor for independent diagnosis among all the pathologists with kappa statistics (±SE) of 0.168 (±0.018). Expert lymphoma pathologists had the highest (albeit only fair) concordance (kappa = 0.373 ± 0.071) and general pathologists the lowest concordance (kappa = 0.138 ± 0.035). Concordance for morphological diagnosis was highest among expert lymphoma pathologists (kappa = 0.356 ± 0.127). Revision of diagnoses after consensus meeting was highest for B-cell lymphoma intermediate between DLB and BL. To conclude, interobserver variation is a significant problem in BL in the post WHO 2008 classification era. Experience with a larger number of cases and joint discussion exercises such as the one we conducted are needed as they represent a simple and effective way of improving diagnostic accuracy of pathologists working in a resource-limited setup.

Madurella mycetoma--a rare case with cranial extension.

BACKGROUND: Madurella species of fungus causes chronic subcutaneous infection of lower extremities; the infection is commonly labeled as Madura foot. We report a case of Madurella infection involving the cranial cavity. Such an involvement by Madurella fungal infection is not recorded in the literature. CASE DESCRIPTION: A 31-year-old non-immunocompromised male patient presented with complaints of left hemifacial pain for 1 year and diplopia on looking toward left side for a period of 2 weeks. On examination, he had ipsilateral sixth nerve paresis. Investigations revealed a large paranasal sinus lesion that extended in the cavernous sinus. The lesion was partially resected. Histologic examination revealed that the lesion was a fungus Madurella mycetomi. CONCLUSION: A rare cranial extension of Madurella fungal infection is reported.

Angiosarcoma of the scalp.

Angiosarcoma is a relatively rare soft tissue tumour. It usually occurs in the head and neck, and especially in the scalp, in elderly people. Its presentation varies from a small plaque to multifocal nodules. The treatment depends on the extent of the disease. Most cases are treated with wide excision with reconstruction. Radiotherapy and chemotherapy are advocated in the recurrent or extensive lesions with regional or distant metastasis. Other modalities such as immunomodulation have been tried. A case of a 55-year-old female patient with a bleeding scalp lesion is presented. Initially thought to be a pyogenic granuloma, on excisional biopsy it was diagnosed as angiosarcoma with microscopic involvement of the margins. Wide excision with reconstruction using a local rotation flap was done at a second stage. The patient was not given postoperative radiotherapy or chemotherapy. There has been no recurrence for two years.

Rare presentation of Kyrle's disease in siblings.

BACKGROUND: Kyrle's disease is a rare variant of primary perforating dermatosis. Its occurrence in a familial setting, especially in children, is extremely uncommon. Similar appearing skin lesions have been described in adults, secondary to metabolic disorders, infective agents as well as exposure to chemicals. We present a rare case of this genodermatosis in two siblings. MATERIALS AND METHODS: Two siblings of a non-consanguineous marriage came with generalized discrete papular lesions with a central keratotic plug. All biochemical and serological investigations were within normal limits. Serial sections of the biopsy revealed typical epidermal invaginations filled with parakeratotic debris and perforation into the dermis with accompanying granulomatous reaction. RESULTS AND CONCLUSIONS: A careful history, detailed routine investigations and serial sections of the skin biopsy are required to demonstrate the typical morphology and stages of evolution of Kyrle's disease. This helps to differentiate the rare primary Kyrle's disease from other primary and secondary keratotic lesions. Due to the familial occurrence, screening of relatives of an index case is recommended.

Asteroid bodies in a lymph node aspirate. A case report.

BACKGROUND: Sarcoidosis, a multisystem disease with tbe potential to occur at any site, has varied clinical manifestations. Cutaneous lesions, seen in one-third of patients, may precede systemic manifestations. Identification of asteroid bodies in aspirated material may aid early diagnosis in the appropriate clinical setting. CASE: A 37-year-old woman had multiple asymptomatic, buff-colored, sucutaneous nodules, 0.5-1.0 cm in diameter, on the scalp, face, dorsum of the wrist and back of the elbows for 2 months. On examination, firm, nontender right inguinal and epitrochlear lymphadenopathy, 2 x 1 cm each, was detected. The epitrochlear lymph node aspirate showed noncaseating epithelioid granulomas, multinucleate giant cells and asteroid bodies. Subsequent biopsy of 1 of the subcutaneous nodules corroborated the aspiration cytology findings. Following the diagnosis of sarcoidosis, other investigations were done. Erythrocyte sedimentation rate was 66 mm at the end of 1 hour, Mantoux test was negative, and chest radiograph showed bilateral reticulonodular shadows. Angiotensin-converting enzyme level was significantly elevated. CONCLUSION: This case draws attention to 1 of the rare cytologic findings of sarcoidosis. Differentiation from other granulomatous lesions, especially when special stains are negative, is difficult. In our case, a lymph node aspirate showing asteroid bodies in a background of granulomatous inflammation supported the diagnosis.