A Rare Cutaneous Malignancy: A Primary Cutaneous Carcinosarcoma with Adnexal Epithelial Component (Trichoblastic Carcinosarcoma) of Left Temporal Region.

Primary cutaneous carcinosarcoma (PCCS) is a rare cutaneous biphasic tumour with just 120 cases being reported till date. The epithelial component trichoblastic carcinoma/ malignant trichoblastoma (trichoblastic carcinosarcoma) is even rarer, so is the present case. An eighty years old male patient presented with a rapidly progressing ulceroproliferative nodular growth on the left temporal region. The incisional biopsy showed primary cutaneous carcinomasarcoma (PCCS). IHC showed epithelial and stromal component marked exclusively for pancytokeratin (AE1/AE3) CK5&6/ p40 and vimentin respectively. Both components were immunopositive for D2-40 and immunonegative for BerEP4/ S100/ Melan A/ MITF, thus final diagnosis given was Trichoblastic carcinosarcoma. Wide excision of the lesion with local rotation flap and skin grafting (thigh skin) was done. Patient remains disease free after four years also. Present case thus highlights the importance of HPE, IHC and early surgery for excellent prognosis of PCCS.

Primary splenic lymphoma: Current diagnostic trends.

The primary splenic lymphoma is extremely uncommon, can present with grave complications like hypersplenism and splenic rupture. In view of vague clinical presentation, it is difficult to arrive at the diagnosis. In such circumstances, histopathological diagnosis is very important. A precise diagnosis can only be made on histopathology and confirmed on immunohistochemistry.Emergency splenectomy is preferred as an effective therapeutic and diagnostic tool in cases with giant splenomegaly. Core biopsy is usually not advised due to a high risk of post-core biopsy complications in view of its high vascularity and fragility. Aim behind highlighting the topic is to specify that core biopsy/ fine needle aspiration cytology can be used as an effective diagnostic tool to arrive at correct diagnosis to prevent untoward complications related to disease and treatment. Anticoagulation therapy is vital after splenectomy to avoid portal splenic vein thrombosis.

Papillary carcinoma of breast: Minireview.

The term "intracystic papillary ductal carcinoma in situ" constitutes only 0.5% to 1% of all breast cancers. It is usually seen in postmenopausal age group. Herein, we are presenting a minireview about this unusual breast malignancy usually difficult to diagnose on clinical grounds and highlighting modalities of diagnosis and management.

Malignant peripheral nerve sheath tumor of proximal third tibia.

A 16-year-old man had aswelling over the anterior aspect of the proximal third of the tibia for 1 year, which was peanut size initially and progressively increased to its present size of 10 cm × 8 cm. He underwent fine needle aspiration cytology (FNAC) twice during this period and reported aspindle cell sarcoma. Malignant peripheral nerve sheath tumor (MPNST) is a malignancy of the connective tissue surrounding the nerves. Previously, MPNST was also known as neurofibrosarcoma, malignant schwannoma, andneurogenic sarcoma. We are reporting this case for its rarity and peculiar mode of presentation. FNAC/core biopsy can be used as an effective tool to achievethe correct pathological diagnosis.

Crohn's disease with gastroduodenal involvement: Diagnostic approach.

Crohn's disease (CD) is a chronic idiopathic inflammatory disease of gastrointestinal tract characterized by segmental and transmural involvement of gastrointestinal tract. Ileocolonic and colonic/anorectal is a most common and account for 40% of cases and involvement of small intestine is about 30%. Isolated involvement of stomach is an extremely unusual presentation of the disease accounting for less than 0.07% of all gastrointestinal CD. To date there are only a few documented case reports of adults with isolated gastric CD and no reports in the pediatric population. The diagnosis is difficult to establish in such cases with atypical presentation. In the absence of any other source of disease and in the presence of nonspecific upper gastrointestinal endoscopy and histological findings, serological testing can play a vital role in the diagnosis of atypical CD. Recent studies have suggested that perinuclear anti-neutrophil cytoplasmic antibody and anti-Saccharomycescervisia antibody may be used as additional diagnostic tools. The effectiveness of infliximab in isolated gastric CD is limited to only a few case reports of adult patients and the long-term outcome is unknown.

Primary signet ring cell carcinoma of the appendix: A rare case report.

Primary adenocarcinoma of the appendix is a rare malignancythat constitutes < 0.5% of all gastrointestinalneoplasms. Moreover, primary signet ring cell carcinomaof the appendix is an exceedingly rare entity. In the present report, we describe a rare case of primary signet ring cell carcinoma of the appendix with ovarian metastasesand unresectable peritoneal dissemination occurring in a 45-year-old female patient. She was clinically misdiagnosed as torsion of ovarian cyst. She underwent appendicectomy and unilateral salpingo-oophorectomy.Histopathology revealed signet ring cell carcinoma and a right hemicolectomy was done. She then received palliative systemic chemotherapy with 12 cycles of oxaliplatin, 5-fluorouracil, and leucovorin (FOLFOX-4). The patient is doing well till today on follow up without progression of disease 10 mo after beginning chemotherapy.

Extremely unusual case of gastrointestinal trichobezoar.

Trichobezoars (hair ball) are usually located in the stomach, but may extend through the pylorus into the duodenum and small bowel (Rapunzel syndrome). Rapunzel syndrome remains uncommon; with fewer than 40 cases reported. To the best of our knowledge, this case may be the first well-documented case with a length of 75 cm. They are almost always associated with trichotillomania and trichophagia or other psychiatric disorders. In the literature several treatment options are proposed, including removal by conventional laparotomy, laparoscopy and endoscopy. Herein, we are reporting an interesting case of an 18-year mentally retarded girl with history of trichotillomania and trichophagia who presented to our emergency department with a history of central abdominal pain associated with vomiting and constipation for five days. An examination showed a trichobezoar requiring emergent surgical intervention, and indicating the need for psychiatric treatment. The trichobezoar was treated successfully by laparoscopy.

Congenital pulmonary airway malformation: A report of two cases.

Congenital pulmonary airway malformation (CPAM), previously known as congenital cystic adenomatoid malformation is a congenital disorder of the lung similar to bronchopulmonary sequestration. In CPAM, usually an entire lobe of lung is replaced by a non-working cystic piece of abnormal lung tissue. This abnormal tissue will never function as normal lung tissue. The underlying cause for CPAM is not known. It occurs in approximately 1 in every 30000 pregnancies. The association between CPAM and malignancy has been well documented. There is a small risk (0.7%) of malignant transformation within the cyst. So early diagnosis and surgical resection is important to prevent the grave complications. Herein, we are reporting two interesting cases of CPAM and one belonged to Type II and other belonged to Type III of Stocker's classification.

Unusual histological variant of malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation.

Malignant peripheral nerve sheath tumor (MPNST) with rhabdomyoblastic differentiation is called as malignant triton tumor (MTT). It is highly aggressive soft tissue tumor with higher local recurrence rate. MTT has poor prognosis than MPNST. MTT seems to be more aggressive in patients with neurofibromatosis (NF-1). We herein, reporting an interesting case of 55 years male with multiple neurofibromas all over the body since 30 years and multiple café-au-lait spots, diagnosed as NF-1. Since 6 years, he had an enlarged mass in left thigh. Wide excision of mass was done. On histopathological examination revealed the diagnosis of MTT and diagnosis of which was confirmed on immunohistochemistry.

An interesting pathological diagnosis - adenoid cystic carcinoma in an adolescent girl.

Adenoid cystic carcinomas (ACCs) constitute 0.1-1 % of all malignant breast tumors. They have better prognosis than other breast malignancies. To date, there have been about 933 cases reported as per English literature. To the best of our knowledge, this case may be the second well-documented case of ACC of breast at younger age.

Primary intestinal lymphangiectasia: Minireview.

Primary idiopathic intestinal lymphangiectasia is an unusual disease featured by the presence of dilated lymphatic channels which are located in the mucosa, submucosa or subserosa leading to protein loosing enteropathy.Most often affected were children and generally diagnosed before third year of life but may be rarely seen in adults too. Bilateral pitting oedema of lower limb is the main clinical manifestation mimicking the systemic disease and posing a real diagnostic dilemma to the clinicians to differentiate it from other common systemic diseases like Congestive cardiac failure, Nephrotic Syndrome, Protein Energy Malnutrition, etc. Diagnosis can be made on capsule endoscopy which can localise the lesion but unable to take biopsy samples. Thus, recently double-balloon enteroscopy and biopsy in combination can be used as an effective diagnostic tool to hit the correct diagnosis. Patients respond dramatically to diet constituting low long chain triglycerides and high protein content with supplements of medium chain triglyceride. So early diagnosis is important to prevent untoward complications related to disease or treatment for the sake of accurate pathological diagnosis.

Epithelial cysts of the spleen: a minireview.

Primary splenic epithelial cyst is an unusual event in everyday surgical practice with about 800 cases reported until date in the English literature. Splenic cysts may be parasitic or non-parasitic in origin. Nonparasitic cysts are either primary or secondary. Primary cysts are also called true, congenital, epidermoid or epithelial cysts. Primary splenic cysts account for 10% of all benign non-parasitic splenic cysts and are the most frequent type of splenic cysts in children. Usually, splenic cysts are asymptomatic and can be found incidentally during imaging techniques or on laparotomy. The symptoms are related to the size of cysts. When they assume large sizes, they may present with fullness in the left abdomen, local or referred pain, symptoms due to compression of adjacent structures (like nausea, vomiting, flatulence, diarrhoea) or rarely thrombocytopenia, and occasionally complications such as infection, rupture and/or haemorrhage. The preoperative diagnosis of primary splenic cysts can be ascertained by ultrasonography (USG), computed tomography or magnetic resonance imaging, although the wide use of USG today has led to an increase in the incidence of splenic cysts by 1%. However, careful histopathological evaluation along with immunostaining for presence of epithelial lining is mandatory to arrive at the diagnosis. The treatment has changed drastically from total splenectomy in the past to splenic preservation methods recently.

Splenic lymphoma with massive splenomegaly: Case report with review of literature.

As per strict criteria of Das Gupta et al, primary splenic lymphoma is very rare. Herein, we are reporting an unusual case of primary large cell splenic lymphoma of B lineage in a middle aged female presenting with massive splenomegaly (3.8 kg) and hypersplenism. After performing therapeutic splenectomy for hypersplenism, a precise diagnosis of diffuse large B cell lymphoma was made on histopathology and confirmed by immunohistochemistry. The patient responded well to standard (Cyclophosphamide, Hydroxydaunorubicin, Oncovin (vincristine), Prednisone or prednisolone) regimen last year and is now in full remission. The splenectomy thereby has prevented the potential grave complications related to hypersplenism and splenic rupture. Our aim behind highlighting the topic is to specify that emergency splenectomy followed by anticoagulation therapy is an effective plan of management to prevent untoward complications related to disease and treatment.

Microscopic colitis: Common cause of unexplained nonbloody diarrhea.

Microscopic colitis (MC) is characterized by chronic, watery, secretory diarrhea, with a normal or near normal gross appearance of the colonic mucosa. Biopsy is diagnostic and usually reveals either lymphocytic colitis or collagenous colitis. The symptoms of collagenous colitis appear most commonly in the sixth decade. Patients report watery, nonbloody diarrhea of a chronic, intermittent or chronic recurrent course. With collagenous colitis, the major microscopic characteristic is a thickened collagen layer beneath the colonic mucosa, and with lymphocytic colitis, an increased number of intraepithelial lymphocytes. Histological workup can confirm a diagnosis of MC and distinguish the two distinct histological forms, namely, collagenous and lymphocytic colitis. Presently, both forms are diagnosed and treated in the same way; thus, the description of the two forms is not of clinical value although this may change in the future. Since microscopic colitis was first described in 1976 and only recently recognized as a common cause of diarrhea, many practicing physicians may not be aware of this entity. In this review, we outline the epidemiology, risk factors associated with MC, its etiopathogenesis, the approach to diagnosis and the management of these individuals.

Isolated gastric Crohn's disease.

Crohn's disease (CD) is a chronic idiopathic inflammatory disease of gastrointestinal tract characterized by segmental and transmural involvement of gastrointestinal tract. Ileocolonic and colonic/anorectal is a most common and account for 40% of cases and involvement of small intestine in about 30%. The stomach is rarely the sole or predominant site of CD. To date there are only a few documented case reports of adults with isolated gastric CD and no reports in the pediatric population. Isolated stomach involvement is very unusual presentation accounting for less than 0.07% of all gastrointestinal CD. The diagnosis is difficult to establish in cases of atypical presentation as in isolated gastroduodenal disease. In the absence of any other source of disease and in the presence of nonspecific upper GI endoscopy and histological findings, serological testing can play a vital role in the diagnosis of atypical CD. Recent studies have suggested that perinuclear anti-neutrophil cytoplasmic antibody and anti-Saccharomycescervisia antibody may be used as additional diagnostic tools. The effectiveness of infliximab in isolated gastric CD is limited to only a few case reports of adult patients and the long-term outcome is unknown.

Unusual case of insitu (intracystic) papillary carcinoma of breast.

The term "intracystic papillary ductal carcinoma in situ", has recently changed and is now more appropriately referred to as "intracystic papillary carcinoma'' constituting only 0.5% to 1% of all breast cancers. Herein, we discuss an unusual case of intracystic insitu papillary carcinoma of breast in a postmenopausal woman, the diagnosis of which was made on histopathology and confirmed by immunohistochemistry. Patient responded well to postoperative adjuvant radiotherapy without any recurrence, thereby preventing further morbidity and mortality related to invasion or tumor progression. So careful histopathological evaluation is the mainstay to arrive at the correct diagnosis to avoid untoward complications related to under diagnosis and /over diagnosis.

Eosinophilic gastroenteritis: an unusual type of gastroenteritis.

Eosinophilic gastroenteritis (EGE) is a rare disorder characterized by eosinophilic infiltration of the bowel wall with various gastrointestinal manifestations. Till date only 280 cases have been described in the literature. A high index of suspicion, by excluding other causes of peripheral eosinophilia, is a pre requisite for accurate diagnosis. EGE is an uncommon gastrointestinal disease affecting both children and adults. It was first described by Kaijser in 1937. Presentation may vary depending on location as well as depth and extent of bowel wall involvement and usually runs a chronic relapsing course. This condition can respond to low dose steroid therapy, thereby preventing grave complications like ascites and intestinal obstruction that might need surgical intervention. The natural history of EGE has not been well documented. Eosinophilic gastroenteritis is a chronic, waxing and waning condition. Mild and sporadic symptoms can be managed with reassurance and observation, whereas disabling gastrointestinal (GI) symptom flare-ups can often be controlled with oral corticosteroids. When the disease manifests in infancy and specific food sensitization can be identified, the likelihood of disease remission by late childhood is high. GI obstruction is the most common complication. Fatal outcomes are rare.

Complications as indicators of quality assurance after 401 consecutive colorectal cancer resections: the importance of surgeon volume in developing colorectal cancer units in India.

BACKGROUND: The low incidence of colorectal cancer in India, coupled with absence of specialized units, contribute to lack of relevant data arising from the subcontinent. We evaluated the data of the senior author to better define the requirements that would enable development of specialized units in a country where colorectal cancer burden is increasing. METHODS: We retrospectively analyzed data of 401 consecutive colorectal resections from a prospective database of the senior author. In addition to patient demographics and types of resections, perioperative data like intraoperative blood loss, duration of surgery, complications, re-operation rates and hospital stay were recorded and analyzed. RESULTS: The median age was 52 years (10-86 years). 279 were males and 122 were females. The average duration of surgery was 220.32 minutes (range 50-480 min). The overall complication rate was 12.2% (49/401) with a 1.2% (5/401) mortality rate. The patients having complications had an increase in their median hospital stay (from 10.5 days to 23.4 days) and the re-operation rate in them was 51%. The major complications were anastomotic leaks (2.5%) and stoma related complications (2.7%). CONCLUSIONS: This largest ever series from India compares favorably with global standards. In a nation where colorectal cancer is on the rise, it is imperative that high volume centers develop specialized units to train future specialist colorectal surgeons. This would ensure improved quality assurance and delivery of health care even to outreach, low volume centers.