Rare Tumors

Metaplastic Breast Cancer (MBC) is a rare group of tumors often presenting as triple-negative. MBC accounts for less than 1% of all breast cancers with the spindle cell variant comprising less than 0.5%. While rare, spindle cell carcinoma is the commonest subtype in the western world. It has a more aggressive biological behavior with increased risk of recurrence and death due to disease compared to triple negative breast cancers. There is no treatment guideline for management of MBC due to the rarity of the disease...

Signet-ring cell carcinomas are an aggressive, poorly differentiated, and highly invasive adenocarcinoma carrying a poor prognosis. Most of these tumors originate in gastrointestinal organs; however, primary lung signet-ring cell adenocarcinomas can rarely occur. Tumoral lymphatic infiltration is a complication of these tumors and can cause phenomena such as lymphangitic carcinomatosis, characterized by a nodular thickening of the pleura, pleural effusions, and mediastinal lymphadenopathies. We report a case of a 63-year-old ex-smoker with a 2-week clinical course of dyspnea and pleuritic chest pain in which a nodular thickening of the pleura and pleural effusion were documented and led to the diagnosis of a primary signet-ring cell adenocarcinoma of the lung with lymphangitic carcinomatosis...

Well-differentiated thyroid carcinoma rarely spreads to soft tissues. Thyroid carcinoma arising within a mature cystic teratoma is even rarer. We report an extremely rare case of synchronous follicular thyroid carcinoma arising within a mature cystic ovarian teratoma and stage IV differentiated thyroid carcinoma. A 62-year-old woman who lived in an iodine-deficient area was accidentally diagnosed with an ovarian cyst during a radiological metastatic work-up for thyroid cancer. Following laparoscopic left salpingo-oophorectomy, histopathological examination revealed a follicular thyroid carcinoma arising within a mature cystic teratoma...

The European Society of Medical Oncology took place in Paris Convention Centre, Paris, France on 9th -13th September 2022 and was attended by more than 28,000 delegates, 23,000 of which were in person and 5000 online. This was the first on-site ESMO congress after the COVID-19 pandemic. This report focuses on a selection of talks delivered at the conference. There was a vast collection of interesting talks, nevertheless, I attended talks that focused on rare cancers.

PURPOSE: Adrenocortical carcinoma (ACC) is a rare and highly aggressive malignant neoplasm, usually diagnosed in advanced stage. Role and efficacy of adjuvant radiotherapy has not been well defined. The objective of this study is to describe the various clinical characteristics and prognostic factors affecting the survival of ACC along with the role radiotherapy on overall survival and relapse free survival. METHODS: A retrospective analysis of 30 patients registered between 2007 and 2019 was carried out...

Papillary breast carcinomas comprise <1% of all breast cancers. They are notorious among surgical pathologists for posing diagnostic difficulty, especially with small sample sizes, such as a core-needle biopsy and carry potential for overtreatment. Solid-papillary carcinoma is a subtype of papillary breast carcinomas that affects elderly females and generally has a favorable diagnosis in its in-situ form. This report focuses on the unique and clinically aggressive presentation and treatment of invasive solid-papillary carcinoma that was discovered along the axillary chest wall after an ipsilateral mastectomy for multifocal ductal carcinoma in situ...

Myxopapillary ependymoma (MPE) is a primary tumor of the central nervous system (CNS), characteristically an indolent malignancy involving the spinal conus medullaris, Filum terminale or cauda equina. We present a rare case of MPE, recurrent in the pelvic soft tissue with eventual pleural and intra-pulmonary metastasis. Refractory to repeated gross resection, adjuvant radiotherapy, platinum-based chemotherapy and temozolomide exploitation of mutant somatic BRCA1 status with the addition of a poly (ADP-ribose); polymerase inhibitor (PARPi) in a novel combination regimen with olaparib-temozolomide (OT) has achieved stable radiological disease after 10 cycles...

The author describes a rare case of giant adenoid cystic carcinoma (ACC) mimicking large paraganglioma with lower cranial nerve palsy. A 60-year-old female presented with a progressive increase in postauricular swelling with unilateral hearing loss, facial deviation, difficulty in swallowing, and hoarseness of voice. MRI brain showed highly vascular infiltrating and osteolytic mass suggestive of large glomus jugulare versus sarcoma. It was completely engulfing the jugular foramen and lower cranial nerves with bony erosion of the jugular foramen and occipital condyle...

Pancreatic mature cystic teratomas are very rare with limited cases found in the literature. These lesions raise a diagnostic challenge and complicate the surgical approach not only because of their anatomic position but also because of their ever-growing size. An elusive diagnosis, usually leads to the operative theatre where surgical resection takes place. We present a rare case of a large pancreatic cystic teratoma extending into the mediastinum in a 29-year-old woman which was succesfully managed with en-bloc distal pancreatectomy and spleenectomy...

Background: Granulosa cell tumor of the ovary is a rare disease and presents with two clinically and molecularly distinct subtypes: the juvenile and the adult type. GCT is considered as a malignant tumor with an indolent course and a tendency toward late recurrence. Purpose : To assess the clinical and paraclinical features, treatment findings, survival outcomes, and explored the prognostic factors in the granulosa cell tumor. Methods : The current study was conducted on 28 GCT patients who had surgical operations and adjuvant chemotherapy (stage IC-IV) by applying a retrospective cohort analysis...

Myxoid liposarcoma (MLPS) is the second most prevalent subtype of liposarcoma. It is usually found in the deep tissues of the lower limbs and rarely in gynecologic tract. Herein we present the second case in the English literature of a primary MLPS arising from the broad ligament which was thought to be a borderline ovarian tumor. The aim is to discuss its clinical and pathological characteristics. A 42-year-old woman presented with pelvic pain for the last 6 months. Magnetic resonance imaging was not specific...

The incidence of malignant tumors diagnosed during pregnancy is increasing, often ascribed to the recently recognized trend that many women are postponing childbirth. Although early diagnosis is optimal for both mothers and fetuses, the diagnosis of malignant tumors during pregnancy is often delayed until an advanced stage, because generalized symptoms of pregnancy and malignancy may overlap, such as shortness of breath, chest or abdominal discomfort. The study patient was 21 years old, and 31 weeks-pregnant when she was diagnosed with primary tracheal adenoid cystic carcinoma (ACC)...

Background: This review will outline the evaluation, diagnosis, and management of dermatofibrosarcoma protuberans and emphasizes multidisciplinary role of nurses, plastic surgeons and radiation oncologist in this recurrent metastatic lesion. It pinpoints affected population at risk, clinical features, and reconstruction options. No analytical research has been done in this area. Material and Methods: A scoping review of patients of DFSP who underwent reconstruction after excision of tumors was performed in the Department of Burn and Plastic Surgery, AIIMS Rishikesh...

Langerhans histiocytosis or Langerhans cell histiocytosis (LCH) is a rare benign pathology representing less than 1% of orbital tumors. It can cause either localized or generalized lesions, leading to the destruction of hard and soft tissues. Eosinophilic granuloma is the most benign form and the predominant clinical presentation of LCH. We report a case of eosinophilic granuloma with orbital involvement in an 18-year-old male patient. Orbital radiotherapy was initially planned, but finally it was not performed due to a spontaneous regression of the lesion after the incisional biopsy...

Metastasis of pleomorphic adenoma (PA) is rare and usually presented as a locoregional recurrence developed many years after excision of the primary tumor although the PA is the most common neoplasm in the parotid gland. We described a case of a 48-year-old male with a parotid tumor with multiple enlarged ipsilateral lymph nodes which suggested a malignancy. The tumors had been neither evaluated nor excised and preoperative evaluation revealed benign PA in both lesions. After the complete surgical excision, the final pathology was notable for benign PA with metastasis to regional lymph nodes...

Inflammatory myofibroblastic tumor (IMT) is an uncommon chest pathology. Treatment primarily focuses on surgical resection for diagnostic and therapeutic purposes. However, there are instances in which alternative therapies with steroids, chemotherapy, or radiation are necessary. We discuss a case of recurrent IMT for which very low dose radiation proved an effective treatment.

Cortical ependymomas are currently not considered a subgroup of supratentorial ependymomas; however, there is a growing body of literature investigating the natural history of these lesions compared to supratentorial ependymomas. We performed a systematic literature review of cortical ependymomas with a focus on the natural history, clinical characteristics, and clinical outcomes of these lesions as compared to supratentorial ependymomas. Our search revealed 153 unique cases of cortical ependymomas. The mean age on presentation was 21...

Ewing sarcoma is a primitive neuroectodermal tumor which seldom presents with primary disease in people over age 40 and outside of the appendicular or axial skeleton. We examine a case of primary thoracic Ewing Sarcoma diagnosed initially by CT-guided biopsy in a woman at the age of 74 years. The disease progressed after initial combined modality therapy consisting of neoadjuvant chemotherapy, surgical resection, and adjuvant radiation therapy and two additional courses of multiagent chemotherapy. After relapse of her disease, subsequent second- and third-line systemic agents which included chemotherapy and targeted agents were given with disease stabilization achieved now over 30 months from initial diagnosis...

BACKGROUND: Schwannomas are mesenchymal tumors arising from neural sheath cells and whose diagnosis is based on immunohistochemistery. The digestive and especially colonic location of this tumor is rare. Commonly described in elderly patient, their malignancy is unusual. CASE REPORT: We report the case of a 23-year-old girl, with learning disability, operated in emergency for acute peritonitis. Peroperatively, we discovered a peritonitis secondary to a bulky perforated cecal tumor...

Pleomorphic hyalinizing angiectatic tumor (PHAT) is a rare tumor of the soft tissue, usually located in lower extremities. There are rarely tumors reported in other anatomic locations. Herein, we report retroperitoneal PHAT in a male patient. A 41-year-old man was referred to our clinic due to an incidentally found retroperitoneal mass. Computed tomography (CT) scan showed a solid hypoechoic lesion containing fat component and calcified elements measuring about 80*72*45 mm in the right lower quadrant (RLQ) of the abdomen...